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Doppler Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014 Children’s Mercy Hospital, KC, MO

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Page 1: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Doppler Assessment in Congenital

Heart Disease

and Fetal Heart Failure

Cristiano Jodicke MD

Fetal Cardiology Symposium

September 13 2014

Childrenrsquos Mercy Hospital KC MO

bull Define fetal congestive heart failure

bull Describe tools used to assess severity of CHF

bull Discuss causes and treatment of fetal hydrops fetalis

Objectives

Objectives

bull Appropriate use of Doppler for fetal evaluation

bull Demonstration of normal fetal Doppler patterns for

umbilical artery middle cerebral artery ductus

venosus and umbilical vein

bull Abnormal fetal Doppler patterns ndash what do they

look like and what does the information mean

bull Application in congenital heart disease

Congestive heart failure

Heart is unable to maintain

sufficient blood flow to meet

the needs of the body

bull Shortness of breath

bull Exercise intolerance

bull Body swelling

Fetal congestive heart failure

LA

Poor tissue perfusion

Acidosis

Fetal

death

Factors affecting perinatal cardiac

output

Stroke volume

Preload

Afterload

Contractility

Parallel

ventricular

ejection

Fetal myocardium

bull Less T-tubular system

bull Less organized myofibrils in the fetus

bull Differences in Ca uptake into the sarcoplasmic

reticulum

bull Decreased sympathetic innervation

bull Fetal myocytes

bull Myocardial growth -gt increase in the number of

muscle cells

smaller in size

mitochondria

sarcoplasmic reticulum

myofilaments

alpha- and beta-adrenoceptors

t-tubuli

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 2: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

bull Define fetal congestive heart failure

bull Describe tools used to assess severity of CHF

bull Discuss causes and treatment of fetal hydrops fetalis

Objectives

Objectives

bull Appropriate use of Doppler for fetal evaluation

bull Demonstration of normal fetal Doppler patterns for

umbilical artery middle cerebral artery ductus

venosus and umbilical vein

bull Abnormal fetal Doppler patterns ndash what do they

look like and what does the information mean

bull Application in congenital heart disease

Congestive heart failure

Heart is unable to maintain

sufficient blood flow to meet

the needs of the body

bull Shortness of breath

bull Exercise intolerance

bull Body swelling

Fetal congestive heart failure

LA

Poor tissue perfusion

Acidosis

Fetal

death

Factors affecting perinatal cardiac

output

Stroke volume

Preload

Afterload

Contractility

Parallel

ventricular

ejection

Fetal myocardium

bull Less T-tubular system

bull Less organized myofibrils in the fetus

bull Differences in Ca uptake into the sarcoplasmic

reticulum

bull Decreased sympathetic innervation

bull Fetal myocytes

bull Myocardial growth -gt increase in the number of

muscle cells

smaller in size

mitochondria

sarcoplasmic reticulum

myofilaments

alpha- and beta-adrenoceptors

t-tubuli

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 3: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Objectives

bull Appropriate use of Doppler for fetal evaluation

bull Demonstration of normal fetal Doppler patterns for

umbilical artery middle cerebral artery ductus

venosus and umbilical vein

bull Abnormal fetal Doppler patterns ndash what do they

look like and what does the information mean

bull Application in congenital heart disease

Congestive heart failure

Heart is unable to maintain

sufficient blood flow to meet

the needs of the body

bull Shortness of breath

bull Exercise intolerance

bull Body swelling

Fetal congestive heart failure

LA

Poor tissue perfusion

Acidosis

Fetal

death

Factors affecting perinatal cardiac

output

Stroke volume

Preload

Afterload

Contractility

Parallel

ventricular

ejection

Fetal myocardium

bull Less T-tubular system

bull Less organized myofibrils in the fetus

bull Differences in Ca uptake into the sarcoplasmic

reticulum

bull Decreased sympathetic innervation

bull Fetal myocytes

bull Myocardial growth -gt increase in the number of

muscle cells

smaller in size

mitochondria

sarcoplasmic reticulum

myofilaments

alpha- and beta-adrenoceptors

t-tubuli

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 4: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Congestive heart failure

Heart is unable to maintain

sufficient blood flow to meet

the needs of the body

bull Shortness of breath

bull Exercise intolerance

bull Body swelling

Fetal congestive heart failure

LA

Poor tissue perfusion

Acidosis

Fetal

death

Factors affecting perinatal cardiac

output

Stroke volume

Preload

Afterload

Contractility

Parallel

ventricular

ejection

Fetal myocardium

bull Less T-tubular system

bull Less organized myofibrils in the fetus

bull Differences in Ca uptake into the sarcoplasmic

reticulum

bull Decreased sympathetic innervation

bull Fetal myocytes

bull Myocardial growth -gt increase in the number of

muscle cells

smaller in size

mitochondria

sarcoplasmic reticulum

myofilaments

alpha- and beta-adrenoceptors

t-tubuli

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 5: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal congestive heart failure

LA

Poor tissue perfusion

Acidosis

Fetal

death

Factors affecting perinatal cardiac

output

Stroke volume

Preload

Afterload

Contractility

Parallel

ventricular

ejection

Fetal myocardium

bull Less T-tubular system

bull Less organized myofibrils in the fetus

bull Differences in Ca uptake into the sarcoplasmic

reticulum

bull Decreased sympathetic innervation

bull Fetal myocytes

bull Myocardial growth -gt increase in the number of

muscle cells

smaller in size

mitochondria

sarcoplasmic reticulum

myofilaments

alpha- and beta-adrenoceptors

t-tubuli

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 6: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Factors affecting perinatal cardiac

output

Stroke volume

Preload

Afterload

Contractility

Parallel

ventricular

ejection

Fetal myocardium

bull Less T-tubular system

bull Less organized myofibrils in the fetus

bull Differences in Ca uptake into the sarcoplasmic

reticulum

bull Decreased sympathetic innervation

bull Fetal myocytes

bull Myocardial growth -gt increase in the number of

muscle cells

smaller in size

mitochondria

sarcoplasmic reticulum

myofilaments

alpha- and beta-adrenoceptors

t-tubuli

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 7: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal myocardium

bull Less T-tubular system

bull Less organized myofibrils in the fetus

bull Differences in Ca uptake into the sarcoplasmic

reticulum

bull Decreased sympathetic innervation

bull Fetal myocytes

bull Myocardial growth -gt increase in the number of

muscle cells

smaller in size

mitochondria

sarcoplasmic reticulum

myofilaments

alpha- and beta-adrenoceptors

t-tubuli

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 8: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal myocardium

bull Reduced ability of

the fetal heart to

contract and

generate force

bull Lower myocardial

compliance

bull Diminished Frank

Starling mechanism

Decreased cardiac

reserve

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 9: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal myocardium

bull Elevated venous pressure (reduce lymphatic flow)

bull Elevated filling pressures

bull Lower albumin concentration

plasma arginine vasopressin (decreases urinary production)

angiotensin II (increases fluid accumulation)

atrial natriuretic peptide (increases capillary permeability)

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 10: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Hydrops

bull Edema with one or more

effusions or effusions in

at least two spaces

Ascites

Pleural effusion

Pericardial effusion

Skin edema

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 11: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Hydrops

Ascites

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 12: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Pleural Effusion

Spine

RV

RA

LA Left Right

Heart

Pleural effusion

Lung

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 13: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Hydrops

Pericardial effusion

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 14: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Hydrops

Skin edema

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 15: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Differential Diagnosis

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 16: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Cardiovascular

Malformation

Left heart hypoplasia

Atrioventricular canal defect

Right heart hypoplasia

Closure of foramen ovale

Single ventricle

Transposition of the great vessels

Ventricular septal defect

Atrial septal defect

Tetralogy of Fallot

Ebstein anomaly

Premature closure of ductus

Truncus arteriosus

Tachyarrhythmia

Atrial flutter

Paroxysmal atrial tachycardia

Wolff-Parkinson-White syndrome

Supraventricular tachycardia

Bradyarrhythmia

High-output failure

Neuroblastoma

Sacrococcygeal teratoma

Large fetal angioma

Placental chorioangioma

Umbilical cord hemangioma

Cardiac rhabdomyoma

Other cardiac neoplasia

Cardiomyopathy

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 17: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Differential Diagnosis

Chromosomal

45X

Trisomy 21

Trisomy 18

Trisomy 13

18q+

13q-

45X46XX

Triploidy

Chondrodysplasias

Thanatophoric dwarfism

Short rib polydactyly

Hypophosphatasia

Osteogenesis imperfecta

Achondrogenesis

Hematologic

α-Thalassemia

Fetomaternal Transfusion

Parvovirus B19 Infection

In Utero Hemorrhage

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Red Cell Enzyme Deficiencies

Thoracic

Congenital Cystic Adenomatoid Malformation of Lung

Diaphragmatic Hernia

Intrathoracic Mass

Pulmonary Sequestration

Chylothorax

Airway Obstruction

Pulmonary Lymphangiectasia

Pulmonary Neoplasia

Bronchogenic Cyst

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 18: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Differential Diagnosis

Twin Pregnancy

Twin-twin transfusion syndrome

Acardiac twin

Infections

Cytomegalovirus

Toxoplasmosis

Parvovirus B19 (Fifth Disease)

Syphilis

Herpes

Rubella

Malformation Sequences

Noonan Syndrome

Arthrogryposis

Multiple Pterygia

Neu-Laxova Syndrome

Pena-Shokeir Syndrome

Myotonic Dystrophy

Saldino-Noonan Syndrome

Metabolic

Gaucher Disease

GM1 Gangliosidosis

Sialidosis

Mucopolysaccharidosis Type 4a

Urinary

Urethral Stenosis or Atresia

Posterior Urethral Valves

Congenital Nephrosis (Finnish)

Prune Belly Syndrome

Gastrointestinal

Midgut Volvulus

Malrotation of the Intestines

Duplication of the Intestinal Tract

Meconium Peritonitis

Hepatic Fibrosis

Cholestasis

Biliary Atresia

Hepatic Vascular Malformations

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 19: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Diagnostic Approach

Maternal history

Ultrasonography

Fetal echocardiography

Labs

Fetal karyotype

Screening for lysosomal storage diseases

TampS

screen for hemoglobinopathies

Kleihauer-Betke

TORCH titers

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 20: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Causes of fetal congestive heart

failure

bull Fetal arrhythmias

bull Anemia

bull Congenital heart disease with valvular regurgitation

bull Non-cardiac malformations such as diaphragmatic

hernia or cystic hygroma

bull TTTS

bull Atrioventricular fistula with high cardiac output

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 21: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Team Approach

bull Perinatologists

bull Cardiologists

bull Neonatologists

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 22: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal heart assessment

Contractility

Cardiac size

Arterial Venous Doppler

Four-valve Doppler

Myocardial perfomance

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 23: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Doppler

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 24: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Arterial Doppler Waveform Analysis

Hemodynamic Basis

Maulik Ultrasound Obstet Gynaecol 1993 Downing Maulik Phillps Kaddado US Med Biol 1993 Maulik Arbeille Kadado Biol Neonate 1992 Downing Yarlagadda Maulik J Ultrasound Med 1991 Downing Yarlagadda Maulik J Ultrasound Med 1991

Maulik

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 25: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Umbilical Artery

Doppler

End Diastolic Flow Maulik (2005) Doppler Ultrasound In ObGyn

2nd Edition Springer

16 wks

20 wks

24 wks

28 wks

32 wks

36 wks

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 26: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Doppler Analysis ndash the Indices

Resistance Index (RI)

Pulsatility Index (PI)

SystolicDiastolic Ratio (SD)

Diastolic Average Ratio (DA)

Maulik et al 1984

Maulik

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 27: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Umbilical Artery Doppler

AbsentReverse End-Diastolic Flow

Compiled from 1126 reported cases

Maulik D Supplement 16 Williams

Obstetrics 19th Ed 1997

Perinatal Outcome Mean

Perinatal mortality 45

Gestational age 316 wks

Cesarean for fetal distress

73

Apgar score at 5min lt7 26

Admission to NICU 84

Congenital anomalies 10

Aneuploidy 64

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 28: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Umbilical Artery Doppler in CHD bull Obstetric complications eg fetal growth restriction

bull Increased abnormality in CHD with

bull Aneuploidy

bull Other malformations

bull Isolated CHD

bull Most cases no specific change

bull Higher UA pulsatility index in right heart

obstructive lesions eg pulmonary stenosis

pulmonary atresia TOF severe Ebsteinrsquos

tricuspid atresia

Copel et al JUM 1999 Ursem et al UOG 2001

Donofrio et al 2003 Kaltman et al UOG2005

Maulik

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 29: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Middle Cerebral Artery Doppler

Arbeille et al UMB 1987

Maulik

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 30: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal Cerebral Artery Doppler in CHD

bull MCA Doppler in hypoplastic left heart

bull Decreased CV flow impedance

bull Lower MCA Doppler indices

bull MCA Doppler in right sided obstructions

bull gtgt left sided obstructive lesions

bull gt normal

bull Cerebral Dopplerumbilical Doppler (CPR UC)

bull Controversial - ldquobrain sparing effectrdquo in CHD

bull Higher (Donofrio 2003) vs normal (Rychik 2005)

bull Predictor of neurological outcome in CHD

Maulik

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 31: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Absent or reverse A wave

bull Right heart dysfunction

bull Late predictor fetal decompensation

bull Independent predictor of mortality in FGR

bull Independent predictor of intact survival (neonatal)

Mavrides et al UOG 2009 Baschat et al UOG 2007 Baschat et al UOG 2009Borell et al UOG 2013

Ductus Venosus Doppler

Kiserud Dop US Ob Gyn

(EdMaulik) Springer 2005

Maulik

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 32: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal Ductus Venosus Doppler in CHD

bull Absent or reverse ldquoArdquo wave indicates fetal cardiac

failure and predicts the outcome including mortality

bull Absent or reverse ldquoArdquo in 1st Trimester predicts 30-

40 of euploid CHDs

bull Increased peak velocities in severe outflow tract

obstructions (HLHS PS AS TOF Ebsteinrsquos TV atresia)

bull Abnormal DV in CHD with other malformations

aneuploidy fetal growth restriction

bull No significant changes in most cases of CHD

Maulik

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 33: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

bull Advanced fetal decompensation

bull Reflects right heart dysfunction with increased right

atrial pressure

bull Poor prognosis

Umbilical Vein Pulsation Maulik

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 34: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Low EA ratio

Mitral valve

Pulsed Doppler of Mitral Tricuspid

Valve

Impaired ventricular diastolic function

Decreased preload

Decreased ability active myocardial relaxation

Valvular dysfunction

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 35: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Tricuspid regurgitation

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 36: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Mitral regurgitation

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 37: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Outflow tracts

Pulmonary Aortic

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 38: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Cardiac output

Stroke Volume x FHR

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 39: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Stroke Volume=V x A

Volume= Velocity time integral (VTI)

Area=Cross-sectional area of the valve

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 40: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Normal Heart Size

Left Right LA

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 41: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Cardiomegaly

Universal

sign of heart failure

CT area ratio= (normal 02 - 035)

CT circumference ratio= normal lt 05

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 42: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Measurements of ventricular wall

thickness

Any left ventricular

posterior wall thickness

greater than or equal to

4 mm is abnormal

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 43: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Myocardial performance index

Tei et al J Cardiol 1995 Dec26(6)357-66

Dujardin et al Am J Cardiol 1998 Nov 182(9)1071-6

Systolic and Diastolic function

bullindependent of heart rate

bull independent of ventricular geometry

bull non invasive

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 44: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Ventricular dysfunction

Prolongation of

Isovolumetric contraction time

(ICT)

Prolongation of

Isovolumetric relaxation time

(RCT)

Reduction of ejection time

(ET)

ICT + IRT

ET

Tei index

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 45: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Ichizuka et al Early Hum Develop 200581273-279

(a- b)

b

How to calculate the Tei

index

Left lt 076

Right lt 065

Normal

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 46: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fractional shortening

End-diastolic dimension minus End-systolic dimension

End-diastolic dimension

Fractional shortening is constant

throughout gestation

(normalgt028)

Myocardial compromise

Increase in the fetal ventricular workload

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 47: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Fetal cardiovascular profile score

Normal -1 point -2 points

CVP score of ge8 is

associated with a good

perinatal

outcome

Huhta et al Ultrasound Obstet Gynecol 2008 31 284ndash288

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 48: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

A cardiovascular profile score in the

surveillance of fetal hydrops Hofstaetter C Hansmann M Eik-Nes SH Huhta JC Luther SL

J Matern Fetal Neonatal Med 2006 Jul19(7)407-13

Human fetal cardiovascular profile score

and neonatal outcome in intrauterine

growth restriction

Maumlkikallio K Raumlsaumlnen J Maumlkikallio T Vuolteenaho O Huhta JC

Ultrasound Obstet Gynecol 2008 Jan31(1)48-54

Cardiovascular profile score in those who died

was 5

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 49: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

Prognosis

Gestational age

Presenting signs and symptoms

Fetal therapy

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 50: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Medical treatment of fetal heart

failure

bull Prolonging pregnancy

bull Preventing prematurity and prenatal asphyxia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 51: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

Consultation with neonatology

Consultation with cardiology

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 52: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

Digoxin

Myocardial dysfunction

Arrhythmias

High output states (fistula

and anaemia)

Cardiomegaly

Decreased ventricular

shortening

Heart failure

will often occur outside 60 and 200 beatsmin

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 53: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

bull Anti-Rho and anti-La

antibodies Valvular regurgitation

Heart block

Myocardial dysfunction

Effusion

Dexamethasone 4 mg daily

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 54: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

Laser treatment TTTS

Acardiac twins

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 55: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

Transfusion Anemia

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 56: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

In utero balloon

angioplasty

Aortic stenosis

Hypoplastic Left Heart

Syndrome and Intact or Highly

Restrictive Atrial Septum

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Marshall A C et al Circulation 2004110253-258

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 57: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Biophysical Profile

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 58: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Management

Delivery time

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

CREASY AND RESNIKS MATERNAL-FETAL MEDICINE PRINCIPLES AND PRACTICE SIXTH EDITION

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 59: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

- Heart failure can be diagnosed in fetuses

and the severity can be estimated

- Serial studies with emphasis on venous

Doppler can be useful in management

- Transplacental treatment of fetal heart

failure could result from accurate diagnosis

and fetalmaternal stratification

Huhta et al Seminars in Fetal amp Neonatal Medicine (2005) 10 542e552

Conclusions

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 60: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Conclusions

bull Fetal Doppler findings in CHD depend on

bull Associated malformations

bull Aneuploidy

bull Obstetrical complications eg fetal growth restriction

preeclampsia

bull Fetal arterial Doppler has selective utility in CHD

bull Fetal venous Doppler has defined utility in CHD

bull Absent or reverse ldquoArdquo wave in the ductus venosus

indicates fetal heart failure and poor prognosis

bull Umbilical venous pulsations indicate poor prognosis

Thank You

Page 61: Doppler Assessment in Congenital Heart Disease … Assessment in Congenital Heart Disease and Fetal Heart Failure Cristiano Jodicke, MD Fetal Cardiology Symposium September 13, 2014

Thank You