topic critical congenital heart disease

Critical Congenital Heart Disease

Sasathorn Tharapoom

Ubolwan Chansingthong 5th year medical student

Advisor

Yasinee Apiraknapanon M.D.

Department of Pediatric, Naresuan University Hospital

Embryology and Physiology

Fetal Circulation

Flow Chart of Fetal Circulation

Neonatal Circulation

1. Placenta Removal 2. Breathing

Lung Expansion

Pulmonary Artery Dilatation

PVR

Ductus Venosus Closure

Flow to RA

RA Pressure

PBF

LA Pressure

Close Foramen Ovale

SVR

Flow to LA

PDA Flow

PaO2

Circulatory Adjustment After Birth

1. Placenta Removal 2. Breathing

Lung Expansion

Pulmonary Artery dilatation

PVR PBF

SVR

PDA flow

PaO2

Closure of Ductus Arteriosus

PGE

Lt. to Rt. Rt. to Lt. Shunt

Ductal tissue contraction

Close Ductus Arteriosus

Human Circulation

Fetal vs. Infant Circulation

• Fetal

• Low pressure system

• Right to left shunting

• Lungs non-functional

• Increased pulmonary

resistance

• Decreased systemic

resistance

• Infant

• High pressure system

• Left to right blood flow

• Lungs functional

• Decreased pulmonary

resistance

• Increased systemic

resistance

Approach to Critical Congenital Heart Disease (CCHD)

Classification of CCHD

• Ductal dependent for pulmonary blood flow

• Ductal dependent for systemic blood flow

• Ductal dependent for blood mixed

• Total anomalous pulmonary venous return with obstruction

Clinical Presentation in CCHD

• Can be asymptomatic in newborn period

• Tachypnea o Many other etiologies

• Absent or weak femoral pulses o Only occurs after the PDA has closed

• Murmur o Occur in up to 77% of newborns, often not pathologic

o Some CCHD lesions do not have murmurs

o Some forms of CCHD do not have murmur until pulmonary vascular resistance falls

• Cyanosis from hypoxia

• Shock

Clinical Presentation in CCHD

Clinical

• Ductal dependent for

pulmonary blood flow

• Ductal dependent for blood

mixed

• Total anomalous pulmonary venous return with obstruction

• Cyanosis from hypoxia • + Tachypnea, heart murmur

• Ductal dependent for systemic

blood flow

• Shock

• Absent or weak femoral pulses

(PDA closed)

• + Tachypnea, cyanosis, heart murmur

Cyanosis • Visual recognition of hypoxia – not easily detected

• Dependent on hemoglobin levels

• Central cyanosis : deoxygenated Hb ≥ 5𝑔/𝑑𝑙

• Peripheral cyanosis : poor perfusion

• Differential cyanosis : preductal SpO2 > postductal

SpO2

• Reversed-differential cyanosis : postductal SpO2 >

preductal SpO2

Cyanosis in Newborn • Heart : CCHD

• Lungs

o Parenchyma : MAS, RDS, pneumonia, atelectasis etc.

o Airway : choanal atresia, Tracheal stenosis, etc.

o Extrinsic lung compression : pneumothorax, pleural effusion,

diaphragmatic hernia, etc.

o Hypoventilation : CNS/neuromuscular disease, sedation,

sepsis etc.

• PPHN

• Abnormal hemoglobin : methemoglobinemia

• Metabolic : hypothermia, hypoglycemia, neonatal

sepsis (poor tissue perfusion)

Initial Evaluation and Management

• ABC : airway, breathing and circulation

• Oxygen supplement : keep SpO2 75-85%

• PGE1

• Pre and postductal SpO2

o Differential cyanosis (preductal SpO2 > postductal SpO2) :

PPHN, severe coarctation of aorta with PDA

o Reverse differential cyanosis (postductal SpO2 > preductal

SpO2) : D-TGA with pulmonary hypertension, D-TGA with

coarctation of aorta

• Hyperoxia test ในผปวยทเขยว

Acute Mx.

• ABC

Hx, PE

• CCHD : heart murmur, cardiomegaly hepatomegaly

• PPHN : respiratory distress, labile cyanosis

Inv.

• CXR

• Hct, blood glucose, ABG

• Hyperoxia test, hyperventilation, pre-post duct PaO2

EKG •Suspected CCHD

PGE1

•Can’t exclude ductal dependent lesion

•Consult & supportive treatment

Test Method Result (ABG) Suggest

Hyperoxia Exposed to 100%

FiO2 for 5-10 min

• PaO2 increases to

>100mmHg


< 20mmHg

• Pulmonary

parenchymal

disease

• PPHN/CCHD

Hyperoxia-

Hyperventilation

Mechanical

ventilator with FiO2

100%

RR 100-150 bpm


>100mmHg

without

hyperventilation

• PaO2 increases at

a critical PCO2

often to <25mmHg

• No increase in

PaO2 despite

hyperventilation

• Pulmonary

parenchymal

disease

• PPHN

• CCHD

Test Method Result (ABG) Suggest

Simultaneous

preductal-

postductal PO2

Compare PO2 of right

arm/shoulder to that lower

abdomen/extremities

• Preductal

PO2 ≥15 +

postductal PO2

• Rt. to Lt.

shunt at

PDA

Central Cyanosis

Hyperoxia test

Not Improved

Hyperoxia-Hyperventilation test

Improved

Improved Not Improved

PPHN CCHD Echo

Pulse Oximetry for CCHD (CCHD Screening)

• Pulse oximetry can identify hypoxemia that is not

visible clinically

• Hypoxemia occurs in many forms of CHD

• Can also identify differences in pre and post ductal

saturations

• Pulse oximetry is quick, easy, and safe

Primary Targets for Screening

• Hypoplastic Left Heart Syndrome

• Pulmonary Atresia

• Tetrology of Fallot

• Total Anomalous Pulmonary Venous Return

• Transposition of Great Arteries

• Tricuspid Atresia

• Truncus Arteriosus

Routine Pulse Oximetry Screening

PROS CONS

• Earlier detection of

CHD, earlier treatment

• Decrease in mortality

• Detection of other

conditions (PPHN, PNX,

PNA, early sepsis)

• Low risk of harm, non-

invasive

• Cost?

• False reassurance if

negative screen

• False Positives

• Parental and staff

anxiety

• Elevated use of

healthcare

• Cost?

Ductal Dependent “Pulmonary Blood

Flow”

Ductal Dependent Pulmonary Blood Flow

• Obstruction to pulmonary blood flow

• most common causes : right-sided obstructive lesion

• Need PDA for pulmonary blood flow

• Presentation o Progressive cyanosis

o PE : single S2 + murmur

o Chest X-ray : decreased PVM

Ductal Dependent Pulmonary Blood Flow

• Common lesions

o Tetralogy of Fallot

o Double outlet right ventricle with pulmonary stenosis

o Single ventricle with pulmonary stenosis

o Pulmonary atresia with intact ventricular septum

o Pulmonary atresia with VSD

o Tricuspid atresia with pulmonary stenosis

Management

• PGE1 0.05-1 mcg/kg/min : increase PBF

• Stabilizes infant : until surgical intervention

• Assisted ventilation and increased environmental

oxygen

• SpO2 75-85% are adequate

PGE1 • Dose 0.05-1mcg/kg/min

o Starting dose of PGE1 is 0.05 mcg/kg/min IV

o If no improvement, ↑ dose to 0.1mcg/kg/min

o usual maintenances dose of PGE1 is 0.025 mcg/kg/min

• Complications of PGE1: o Apnea : direct effect of PGE1 on the CNS

• Need assisted ventilation immediately after PGE1 has been started

o Hypotension : peripheral vasodilation, decreased cardiac output

• continuous measurement of arterial blood pressure

o Fever

o Irritability, abnormal EEG and seizures

o Diarrhea

o hypertrophy of gastric mucosa : longer term effects

Tetralogy of Fallot (TOF)

TOF

• 5-10% of Congenital heart disease

• Most common cyanotic heart disease

• Conotruncal maldevelopment

• Anterior displacement of conus septum

LV

RV

Conus Cordis RVOT, LVOT

1

1

1

0.4 0.6

0.6

0.6

1

Qp : Qs

0.6 : 1

Left heart

Decreased volume

Normal pressure Right heart

Normal volume

Increased pressure

TOF o RVOT obstruction

o RV pressure ~ LV pressure

o Rt. to Lt. shunt (through VSD) cyanosis

• Severity depends on : RVOT obstruction, collateral

vessel

Signs & Symptoms

• Central Cyanosis

• Dyspnea on exertion

• Squatting position

• Clubbing fingers

• Severe cyanosis : tachypnea, single S2 (severe PS)

• SEM at mid LUSB (turbulent flow ผาน RVOT)

• No VSD murmur (pressure RV ~LV)

• Hypoxic spells : severe cyanosis, hyperpnoea, PS murmur เบาสนลง

• Acyanotic TOF (pink TOF) : heart murmur

Vicious Cycle of Hypoxic Spell

(Systemic vascular resistance)

Investigation

• Chest X-ray

• EKG

• Echocardiogram

• Cardiac catheterization

CXR

• Pulmonary

vascular marking

• Boot shaped heart

(RVH)

Right axis deviation,

RVH : Tall R wave with upright T wave in right chest lead,

Deep S wave in left chest lead

Echocardiogram • VSD

• Overriding aorta

• RVOT obstruction : infundibular stenosis/valvular PS

• Turbulent flow

• peak flow velocity pressure gradient

• Main pulmonary artery and bifurcation

characteristics

• Coronary artery abnormality

• Others abnormality : ASD, PDA, collateral vessels

Cardiac Catheterization • For surgery

• Distal pulmonary artery, collateral vessels, abnormal

coronary artery

• Hemodynamic

o Ascending aorta : desaturation

Treatment • Medical treatment : hypoxic spell

• Surgery repair anatomical defect : indication

o Decreased exercise tolerance

o Increased hypoxic spells

o Polycythemia

Hypoxic Spells Management 1. Calm down

2. Knee chest position ( systemic vascular resistance , systemic venous return)

3. Oxygen supplement

4. MO 0.1 mg/kg ( systemic venous return)

5. IV Fluid

(correct dehydration)

6. NaHCO3 1-2 mEq/kg ( acidosis when spell >10-15 min)

7. Propranolol 0.1mg/kg + NSS up to 10 ml IV in 5-10min ( infundibular spasm)

8. Correct anemia 9. Phenylephrine HCl

10. Propranolol 1-2MKday o 3-4 times/day IV (prevent spell)

Surgical Management • Severe cyanosis (Hct >65%) : palliative surgery

(systemic to pulmonary shunt)

o At age 6 month echocardiogram, cardiac catheterization ประเมนขนาด PA

o ถา PAขนาดใหญพอ total correction/เลกท า shunt เพม

• Moderate cyanosis : พจารณาผาตดตามขนาด PA

• Mild cyanosis : total correction (สามารถท าไดเนองจาก PA มขนาดใหญพอ) o VSD closure

o ขยายทางเดนเลอดจาก RV Lungs

Systemic to Pulmonary Shunt

Pulmonary Atresia with VSD

Pulmonary Atresia with VSD

• Extremely severe form of TOF

• Incomplete septation conus cordis VSD

• Abnormal septation of truncus arteriosus atretic

pulmonic valve, abnormal pulmonary trunk

• 70% PBF ผาน PDA, 30% PBF จาก systemic collateral

artery

Lung bud

Paired dorsal a.

Intersegmental a.

Pul. vas. plx. 6th aortic arch

Pulmonary trunk

RPA,LPA

PA

Bronchial a. Dorsal a. collateral

Pulmonary Trunk Abnormalities

PV annulus

Hypoplastic

pulmonary trunk

Systemic Collateral Artery

Signs & Symptoms • Cyanosis and dyspnea in a few hours/days after

birth

• O2sat 90-92% (nonsevere hypoxia)

• Single S2

• No murmur

• SEM/continuous murmur of PDA at LUSB

Investigation • EKG : right axis deviation, RVH

• CXR : decreased PBF, concave/flat pulmonary trunk

• Echocardiogram :

o PDA, collateral a.

o VSD

o Overriding aorta

o ไมพบ pulmonary outflow จาก RV

o Atretic/hypoplastic pulmonary trunk

o Cardiac catheterization : collateral artery

Treatment • Severe cyanosis

o PGE1 0.05-1 mcg/kg/min IV 0.01-0.03 mcg/kg/min

o Systemic to pulmonary shunt/PDA stenting

• Surgery o Palliative surgery 2-3 ปกอนท า total correction

o Total correction : at age 3-5 year

• VSD closure

• Ligate systemic to pulmonary shunt

Double Outlet Right Ventricle (DORV) with

Pulmonary Stenosis

DORV • Cyanotic heart disease

• 0.8-1% of congenital heart disease

• Aorta and pulmonary artery arise from RV

• No arteries arise from LV

DORV • Conotruncal maldevelopment

• Incomplete septation of conus cordis

• the aortic and mitral valves are separated by a

smooth muscular conus

• No leftward shift of LVOT structure

Subaortic

VSD Subpulmonic

VSD

Doubly

committed

VSD

Remoted

VSD

Subaortic

VSD

คลาย Large VSD (Left to right shunt มาก) เขยวไมมาก

O2sat 90-96%

Heart failure + PHT

คลาย d-TGA with VSD เขยวมาก

Heart failure + PHT

Subpulmonic

VSD

Doubly

committed

VSD

Remoted

VSD

คลาย Large VSD

เขยวมากกวา subaortic นอยกวา subpulmonic Heart failure + PHT

PBF

PS

Signs & Symptoms DORV/PS • Hypoxia cyanosis dyspnea

• Severity depends on : severity of PS, relationship

with VSD

• PE :

o Single S2

o SEM at LUSB (PS murmur) may transmit to back

Investigations • CXR

o Normal heart size

o Decreased pulmonary vascular marking as in TOF

• EKG

o Right axis deviation

o RVH ± RAE (in severe PS)

• Echocardiogram

o Relationship of VSD and great arteries

o No LV outflow

o VSD

o Turbulent flow ผาน subvalvular, pulmonary valve

Treatment • Treat hypoxic spell

• Surgery

o Small PA, severe PS, severe cyanosis

• Palliative Blallock-Taussig shunt correction surgery

o Subaortic VSD/doubly committed VSD

• ปด VSD ดวย patch/graft

• แกไข PS (เหมอน TOF)

o Subpulmonic VSD/remote VSD

• ปด VSD แบบ intraventricular tunnel

• Homograft valve conduit ระหวาง RVOT, MPA

o DORV with PS with restrictive VSD

• ขยาย VSD intraventricular tunnel repair

Tricuspid Atresia with Pulmonary Stenosis

Tricuspid Atresia with Pulmonary Stenosis

• SVC/IVC RA TR obstruction need

associated lesion RV

• Associated lesion

o PDA

o PFO

o ASD

o VSD

o Others : TGA, PS

.

Normal related Great arteries

d-TGA

PA PS No PS

Sings & Symptoms • Severity depends on PBF (ถามากจะเขยวนอย)

• Cyanosis and dyspnea in 1st month after birth

• Hepatomegaly

• Heart sound :

o Single S2

o SEM at LUSB (PS/PDA/relative PS)

o SEM mid LSB (restrictive VSD)

Investigation

• Chest X-ray

o Prominent right atrial border

o Decreased pulmonary vasculature

• EKG

• Echocardiogram o RAE, PFO/ASD, atrial septum โปงไปทางซาย

o Hypoplastic RV

o Tricuspid valve ไมมรเปดลงไป RV

EKG

Left superior QRS axis

RAE, LVH

Treatment • Supportive treatment

• Treat hypoxic spell

• PGE1

• เลอดไปปอดนอย (PA/PS), เขยวมาก : systemic to pulmonary

shunt

• Age 6 month

o Cardiac catheterization : PVR, PAP evaluation

o Bidirectional superior cavopulmonary connection (Bidirectional Glenn shunt) : SVC PA

• Age 3 year : Fontan type operation : SVC, IVC PA

Pulmonary Atresia with Intact Ventricular Septum (PA/IVS)

PA/IVS

• 1% of CHD

• Well-formed RV,

tricuspid valve

• Inflammatory process at pulmonic valve

fibrosis

• Hypoplastic RV

• Need PDA, PFO

• Tripatite : TR รวมบอย

• Severity : PDA, PVR

2. Outlet

1. Inlet

3. Trabecular

DA

RVO obstruction

RV systolic

pressure

แรงดนยอนกลบจาก RV ไปCoronary sinusoid

pressure injury effect

to coronary a.

coronary a.:

Intimal, medial

thickening

Proximal coronary

a. stenosis

Myocardium

Sings & Symptoms • Cyanosis and dyspnea in a few hours/days after

birth

• Normal lung sound

• Hepatomegaly (flow ผาน PFO ไมสะดวก RA pressure สง)

• Heart sound :

o Single S2

o Most no murmur

o Systolic murmur (flow ผาน PDA ทก าลงจะปด)

o PSM/SEM of tricuspid regurgitation นอย

CXR

• Normal heart size

• Decreased PVM

• Concave

Pulmonary trunk

• Prominent RA

border

Left axis deviation, RAE, LVH

Echocardiogram • RAE

• Hypoplastic RV

• Atrial septum โปงไปทาง LA

• Right to left shunt ผาน PFO

• TR

• Coronary sinusoid

• Ventriculocoronary artery connection in RV

• PA, small MPS/RPA/LPA, PDA

• Right to left shunt ผาน PDA

Treatment • PGE1 0.05-1 mcg/kg/min

• Definitive repair : เลอกตามลกษณะ RV

o Biventricular repair

o Univentricular repair

o One and a half ventricle repair

o Transplantation

• Catheter intervention o Tripatite, tricuspid valve, RV ขนาดใหญพอ

Single Ventricle with Pulmonary Stenosis

Single Ventricle

• 1% of CHD

• ความผดปกตของการเคลอนยาย AV canal

• Double inlet ventricle

Signs & Symptoms • Hypoxia cyanosis dyspnea

• Severity depends on : severity of PS

• PE :

o Single S2

o SEM at LUSB (PS murmur)

Investigation • CXR

o Normal heart size

o Normal/decreased pulmonary vasculature

• EKG

o Superior axis deviation (common AV valve)

o Ventricular hypertrophy

o Abnormal Q wave

• ECHO

o Ventricular morphology

o AV valve

o Associated cardiac anomaly : e.g. PS, PA

Right superior axis

Deep S in V1, V2, V4

Deep Q in V5, V6

Treatment • PGE1

• Surgery o Systemic to pulmonary artery shunt

o Bidirectional Glenn operation at 6-8 month

o Fontan type operation at 3-5 year

Ductal Dependent “Systemic Blood Flow”

Ductal Dependent Systemic Blood Flow

• Critically obstructed systemic circulation

• Neonate who present with signs of poor systemic

circulation need to be rapidly evaluation and

categorized as having CHD or other diagnosis such

as sepsis or metabolic disease

• Neonate with congenital obstructive left heart

syndrome or left ventricular outflow tract will usually

present in the first few weeks of life as their ductal

dependent systemic circulation become compromised with the closure of the PDA

Ductal Dependent Systemic Blood Flow

• Finding in neonates with cardiogenic shock

o Tachycardia

o Hypotension

o Diaphoresis

o Poor perfusion (dusky, mottled)

o Oliguria

o Acidosis

Management • Maintain an airway (they can quickly tire and

apnea)

• Place the patient on oxygen and cardio-pulmonary

monitoring (be sure to have a bag valve mask and

oxygen already flowing)

• Vital signs should include pre & post ductal pulse

oximetry and blood pressure

• Immediate two IV/IO access points

Management • When concerned about cardiogenic shock only 10

mL/kg of crystalloid should be initially be given

• EKG and portable CXR should be done as soon as

possible

• Bedside initial lab : glucose, CBC, basic metabolic

panel, blood culture and ABG

• Hyperoxia test

Management • Neonate who thought to have a ductal dependent

lesion is to immediately start an infusion of PGE1 to

re-open/maintain the ductus 0.05-0.1 mcg/kg/min

IV route

o Major side effect is apnea

• If not impossible to immediately rule out septic

shock, antibiotics should be given as soon as

possible. With plan LP as soon as the pt. is stabilized

• Consult pediatric cardiologist and echocardiogram

Differential Diagnosis

• Aortic stenosis

• Coarctation of the aorta

• Interruption of the aortic arch

• Hypoplastic left heart syndrome (HLHS)

Aortic Stenosis

Critical Aortic stenosis in NB

เลอดไป aorta นอย LA pressure สง

Pulmonary congestion

เลอดบางสวนผาน foramen ovale

ม ductus arteriosus เลอดจาก Pulmonary artery จงไป aorta ได

ทารกจงม cardiac output เพยงพอ ไมเหนอย

Rt to Lt shunting

Clinical Manifestation

• Critical AS in neonate

o Tachypnea

o Irritable

o Poor feeding

o Cold extremity

Physical Examination

• Systolic thrill at RUSB, suprasternal notch, carotid

artery both side

• SEM at LUPSB, RUPSB radiate to neck

• Valvular AS : systolic ejection click จาก stenotic aortic

valve

• Supravalvular AS : BP Right arm > Left arm and lower

extremities (Coanda effect)

• Subvalvular AS : hypertrophic valve cusp Aortic regurgitation diastolic blowing murmur at LUPSB

Investigation

• ECG:

o Non severe AS : normal EKG or LVH

o Severe AS : LVH and strain pattern

• Chest X-ray

o Cardiomegaly

• Echocardiogram


Treatment • Valvular AS :

o Asymptomatic Mild Moderate AS : observe

• if doppler echo peak pressure gradient ≥ 70 mmHg or

• peak velocity > 4.2 m/s

o Severe valvular AS

• Start with balloon aortic valvuloplasty

• Surgical if cannot or fail cardiac cath

Cardiac

catheterization

and balloon aortic valvuloplasty

Indication for Balloon aortic valvuloplasty

1. critical AS in neonate

2. peak gradient > 60 mmHg

3. Symptoms (angina,

syncope, and/or dyspnea on

exertion) and a transvalvular peak-

to-peak aortic valve gradient at

catheterization greater than 50

mmHg

Treatment • Supravalvular AS and subvalvular AS : only surgery

• Valvular AS :

o Normal size of aortic annulus : open aortic valvulotomy

under inflow occlusion or under cardiopulmonary bypass

o Small aortic annulus : Ross-Konno or Ross’s operation

• Supravalvular AS :

o Dilate ascending aorta under cardiopulmonary bypass

• Subvalvular AS : o Myectomy

Coarctation of The Aorta

Development of Coarctation


• Abnormal development of left 4th and 6th aortic arches

• No real impact prior to birth due to presence of PDA unless there is fetal closure


Ductus tissue theory Hemodynamic theory

• Due to a migration of ductus smooth muscle cells into the periductal aorta with subsequent constriction and narrowing of the aortic lumen

• Evident when ductus closes

• Reduced intrauterine

blood flow causes

underdevelopment

of aortic arch

• Results from reduced

volume of blood flow

through the fetal

aortic arch and isthmus

Pathophysiology

Fetus LV pressure=RV

pressure

Due to ductus arteriosus

Neonate ductus arteriosus

closed

LV pressure increase

LV pressure overload

proximal artery HT

Upper ext. hypertension

Lower ext. hypotension

Ductus closure

LV pressure overload

: immediately

Pulmonary

congestion

low output : distal to

coarctation

Severe HF with

oliguria


• Asymptomatic

o Persistent patent ductus arteriosus (PDA)

o Not severe coarctation

• Pale, irritable, diaphoretic, and dyspneic with absent femoral pulses

• Heart failure and/or shock when the PDA closes

o Severe coarctation


• Absent or delayed femoral pulse (when compared

with the brachial pulse).

• Murmur

o May associated with other cardiac defects

• Differential cyanosis

o Severe coarctation of the aorta

o Large PDA with a right-to-left shunt into the descending

thoracic aorta

• Hepatomegaly

Investigation

• ECG:

o Isolated coarctation normal EKG or LVH

o Coarctation with VSD and PDA RVH or combined

ventricular hypertrophy

• Chest X-ray

o Cardiomegaly

o Figure of 3

o E sign or reverse 3 sign (Barium swallow)

• Echocardiogram


Treatment • Medical treatment

o Clinical heart failure : inotropic drug, diuretic drug before surgery

• Surgical treatment o Asymptomatic isolated coarctation if hypertension

• Coarctation repair

• Balloon angiography

• Coarctation stenting

o Coarctation with VSD

• small VSD : coarctation repair alone

• Large VSD : coarctation repair with pulmonary artery banding

• Staged repair

Interrupted of The Aortic Arch

LV Prox. IAA

RV PA PDA Distal IAA

O2 sat upper ext. > lower ext.

PDA closure =>

Decrease

pulmonary vascular

resistant

Increase pulmonary

blood flow BP upper>lower ext.

Large VSD RV pressure = LV pressure

PDA closure

Decrease pulmonary

vascular resistant

Increase pulmonary

blood flow

Heart failure and

Pulmonary congestion

Lower extremities - Low output

- Acidosis

- Oliguria


• When PDA was closed interrupted aortic arch may

present with

o Dyspnea

o Tachypnea

o Cold extremities


• Tachypnea

• Dyspnea

• Tachycardia

• Weak or absent femoral pulses

• Differential cyanosis

• Crepitation both lung

• S2P loud

• SEM grade 2-3 at mid left sternal border

• Hepatomegaly

Investigation

• EKG

o RVH (upright T wave and tall R wave)

• Chest X-ray

o Cardiomegaly

o Increased pulmonary vascular marking

o Pulmonary venous congestion

• Echocardiogram




• Surgical treatment

Hypoplastic Left Heart Syndrome (HLHS)

LA PFO RA

RV PA PDA aorta

Mixed blood

PDA closure

Decrease pulmonary

vascular resistant

Increase pulmonary

blood flow

Decrease systemic

blood flow

Low output&Shock

Clinical Manifestation – With ASD

• Typically have a "honeymoon" period (adequate

systemic perfusion)

• As the PDA begins to close, infants become

symptomatic

o Systemic perfusion : diminished peripheral pulses

o Pulmonary blood flow hypotension, acidosis, and

respiratory distress

o Symptoms can rapidly progress from cyanosis, increased

respiratory distress, and poor feeding to heart failure and

cardiogenic shock

Clinical Manifestation – Without ASD

• Severe cyanosis and respiratory distress

• Quickly develop cardiogenic shock and die

Physical Examination • Tachypnea

• Dyspnea

• Tachycardia

• Weak or absent peripheral pulses

• Hypotension

• Blood pressure upper = lower ext.

• Mild cyanosis

• Crepitation both lungs

• Active precordium

• Single and loud second heart sound

• SEM grade1-2 at left sternal border

• Hepatomegaly

Investigation

• EKG

o RVH (m/c) or LVH (rare)

• Chest X-Ray

o Cardiomegaly

o Pulmonary venous congestion

• Echocardiogram




o Avoid oxygen if no indication (can cause pulmonary

vasodilation)


o The three steps consist of the following procedures:

• Norwood procedure

• Bi-directional Glenn operation

• Fontan operation

Ductal Dependent “Mixed Blood Flow”

D-TGA

RT Systemic Lung LT

Parallel circulation

Aorta PA

PDA

ASD

VSD

I-TGA

RA LA

Corrected TGA

Aorta PA LV RV

Systemic Lung

Decrease pulmonary

vascular resistant

Decrease PA pressure

Aorta PDA PA

Lung

Oxygenated blood LA

If PDA closure

Progressive cyanosis

LA PFO RA RV

aorta systemic

VSD

Ventricular systole Rt Lt

Ventricular diastole Lt Rt

Bidirectional flow by VSD


• Cyanosis

• Tachypnea

• Signs of congestive heart failure


• Simple TGA

o Tachypnea with cyanosis

o Single S2

o SEM at LUPSB

• TGA with VSD

o Tachypnea with cyanosis

o Single S2

o SEM at LUPSB

o Crepitation both lungs (heart failure)

Investigation

• EKG : RVH

• Chest X-ray

o Cardiomegaly

o Pulmonary venous congestion : increase pulmonary

vasculature

o Egg on a string

Investigation

• Echocardiogram



o If cyanosis after administer PGE1

o Balloon atrial septostomy (BAS)

o Clinical heart failure

• Inotropic drug

• Diuretic drug

Treatment


o All patient

o Atrial switch operation

(ASO)

Total Anomalous Pulmonary Venous Return (TAPVR)

Lung bud

Intraparenchymal pulmonary v. เชอมกบ

common pulmonary v. (ยนจาก LA)

• Splanchnic plexus lung bud

• Venous drainage : Umbilico -

vitelline & cardinal venous

system

Pulmonary vascular bed

• Pulmonary plexus & splanchnic

plexus obliterate • Pulmonary venous drainage ตอกบ

common pulmonary vein

Common pulmonary vein รวมกบ LA

Pulmonary vein เทเขา posterior wall ของ LA

TAPVR

Cor triatrium

Cardiac type Cardiac type Supracardiac

Infracardiac

Mixed

Signs & Symptoms • TAPVR without pulmonary venous obstruction

o แรกคลอดเหมอนปกต แต SpO2 90%

o 3-6 month : เขยว หายใจเรวแรง น าหนกขนนอย ตดเชอทางเดนหายใจบอยๆ

o Mild cyanosis, undernourished, tachypnea, dyspnea

o Tachycardia bulging precordium with palpable right

ventricular impulse

o Widely fixed split S2 , louder S2P, SEM gr 2-3/6 at LUSB, S3

gallop + diastolic rumbling murmur at LLSB

o hepatomegary

Signs & Symptoms • TAPVR with pulmonary venous obstruction

o เขยว หายใจเหนอยเมอแรกคลอด

o Moderate - severe cyanosis, tachypnea with retraction

o Lungs: crepitation

o Loud S2P ± murmur (Systolic murmur at LSB)

o Hepatomegaly

CXR

• TAPVR without

pulmonary venous obstruction o Cardiomegaly

o Increased PVM

EKG • TAPVR without pulmonary venous obstruction

o RVH : rSR’/ tall R in right chest lead

o RAE

• TAPVR with pulmonary venous obstruction

o RVH : T upright in V4R, V1/ tall R in right chest lead

Echocardiogram • RAE

• RVH

• Atrial septum โปงไปทาง LA

• ไมพบ pulmonary vein เทเขา LA แตเทเขา SVC/IVC/Coronary

sinus

• Right to left flow ผาน PFO

• Pulmonary venous confluence ผานเขา systemic vein

Treatment • Medical treatment : ควบคม heart failure

o TAPVR without PVO : digitalis, diuretic

o TAPVR with PVO, pulmonary edema : ETT on ventilator ให

O2, PEEP

• Surgical treatment o เชอม pulmonary vein & LA โดยให anastomosis กวางทสด

o TAPVR with PVO : ควรผาอยางรบดวน

o TAPVR without PVO : ผาเมอผปวยพรอม ทงไวนานจะ heart fail รนแรงขน

Reference

topic critical congenital heart disease

Health & Medicine