cvs simple approach to chd

1

DIAGNOSIS OF CHD

Dr. S. Aswini Kumar. MD

Professor of Medicine

Government Medical College Hospital

Thiruvananthapuram

2

RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

NORMAL

3

History & Symptomatology

Mother’s marriage?

Consanguinity?

Age of Child Birth?

Antenatal History?

Natal History?

Postnatal events?

Physical development?

Mental development?

Breathlessness?

Exertional Syncope?

Cough with sputum?

Bluish discoloration?

History of squatting?

Cyanotic spells?

Pedal edema?

Hemoptysis?

4

Questions to be answered

Anatomic and Functional?

Acyanotic or Cyanotic?

Pulmonary Artery Blood flow?

Malformation of right or left?

Dominant Ventricle?

Pulmonary Hypertension?

What is the hemo-dynamics?

Feasibility of surgical correction?

Finally which CHD is it?

ASD VSD PDA AS PS

COA RSOV CAVF ALCAPA TOF

PA TA EM ASD EM VSD EM PDA

EA cTGA CcTGA PAVF DC

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Planning for investigations

Electrocardiogram

Chest X Ray

Trans-thoracic Echocardiography

Trans-esophageal Echocardiography

Color Doppler Imaging

Magnetic Resonance Imaging

Hemo-dynamic Studies

Angiographic Studies

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Diagnosis of Congenital Heart Disease

CHD

Acyanotic

LR shunt

Atrial

Ventricular

Ductal

Obstructive

Aortic

Pulmonary

Aortic arch

Ao-RtHt Shunt

Ao-RA

Ao-CoSi

LCA-PA

Cyanotic

With PS

4 defects

Valvular

Tricuspid

With PAH

PAH in ASD

PAH in VSD

PAH in PDA

With Normal PAP

EA

cTGA

PAVF

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Acyanotic

Frequent Respiratory Infections

Bulging precordium

Hyperkinetic precordium

Tendency to sweat

Tendency to CHF

Shunt murmur

Flow murmur

CXR: Plethoric lung fields

ASD

•30-50 age

•FemaleVSD

•5-15 aged

•MalePDA

•1-5 aged•child

L-R SHUNT

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ACYANOTIC L-R SHUNT

Normal pulse and BP

Normal JVP and waves

Displaced apex beat

Left Para-sternal Heave

Wide fixed split of S2

Pulmonary ESM

Tricuspid Diastolic flow

murmur

ECG – RAD RVH rSR’ in V1

CXR: CE+ RAE RVH Pul plethora

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Patent foramen ovale - allows a continuation of the atrial shunting of blood, in 25% of people

RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

ASD

Hemodynamics in ASD:

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In ASD there is fixed splitting of S2, with no ventilatory variation of the interval between A2 & P2.

In PS, there is an ejection sound (c), P2 is diminished, A2 and P2 interval with inspiration

In idiopathic dilatation of the pulmonary artery, there is an ejection sound, but P2 is preserved.

Left precordial bulge in a patient with congenital Atrial Septal Defect

Produced by right ventricular enlargement occurring during the growth period.

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ostium secundum (OS) type lies within the fossa ovalis

ostium primum (OP) atrial communication at the endocardial cushion

An SVC Sinus venosus (SV) defect within RA at junction of SVC and PV

An IVC sinus venosus (SV) defect at the junction of RA with IVC

A communication through the coronary sinus (CS)

Five potential spaces of inter-atrial communication

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Development of IA septum

Septum primum

Ostium primum

Septum secundum

Ostium secundum

Foramen ovale

Fossa ovalis

Endocardial cushion

Interventricular foramen

AV Valves

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ECG in Ostium Primum ASDAbnormal LAD of QRS & Incomplete RBBB

ECG in Sinus Venosus ASD

LAD RBBB RVH and I0

Heart Block

ECG in Ostium Secundum ASDRAD RVH Incomplete RBBB

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CXR: CE RAE RVH Plethora

Doppler ECHO showing shunt

Diagrammatic representation

Necropsy specimen - ASD

Cardiac cathetrization

Contrast injection LA-RA

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ACYANOTIC L-R SHUNT

High volume NC pulse

Normal BP and JVP


LV type apical impulse

Systolic thrill at the LSB

Pansystolic murmur

Mitral diastolic flow murmur

LAE LVH in ECG

CXR: CE+ LV+ Pul Plethora

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A defect in the ventricular septum, the wall dividing the left and right ventricles of the heart

The membranous portion, which is close to the atrio-ventricular node, is most commonly affected

inferior muscular portion is less commonly involved

RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

VSD

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I = inlet component, extending from tricuspid annulus to attachments of tricuspid valve

T = trabecular septum, extending from the inlet to the apex and up to the smooth-walled outlet

O = outlet septum or infundibular septum

a = outlet defect; b = papillary muscle of the conus; c = perimembranous defect; d = marginal

muscular defects; e = central muscular defects; f = inlet defect; g = apical muscular defects

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Necropsy (when dead) in VSD

1. Tiny membranous VSD

2. Nonrestrictive perimembranous VSD

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ACYANOTIC L-R SHUNT

High volume collapsing

pulse

Wide pulse pressure



Systolic or continuous

thrill

Train in tunnel murmur

Multiple clicks

Mitral diastolic flow murmur

LAE and LVE in ECG

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A congenital disorder in heart wherein a neonate's ductus arteriosus fails to close after birth

It can be idiopathic (i.e. without an identifiable cause), or secondary to another condition

Contributing factors in humans include: - Preterm birth - Congenital rubella syndrome - Down syndrome

RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

PDA

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Chest X-Ray in PDA

severe pulmonary hypertension, and reversed shunt

arrows indicate the enlarged pulmonary artery segment

Diagrammatic representation of PDA

shunt of oxygenated blood

from aorta (AO) through PDA into PA

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THE FETAL CIRCULATION

arrows indicate the directions of flow.

A fraction of umbilical venous blood enters the ductus venosus and bypasses the liver.

This relatively highly oxygenated blood flows across the foramen ovale to the left heart,

This blood preferentially perfuse the coronary arteries, head, and upper trunk.

The output of the right ventricle flows preferentially across the ductus arteriosus

This circulates to the placenta, as well as to the abdominal viscera and lower trunk.

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Echocardiogram in PDA

Two-dimensional Doppler echocardiogram

left, parasternal short-axis view and right, suprasternal view.

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Aortogram in PDA

Lateral aortogram from a 7-year-old girl with PDA measuring 2 mm at the narrowest point

A 5-cm coil was placed, with no residual flow evident by color Doppler imaging at 24 hours.

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Acyanotic

Exertional dyspnoea and syncope

Normal or low systolic BP

Normally placed forceful or heaving apex

Systolic thrill and Ejection Systolic Murmur

Delayed component of second sound

ECG - Ventricular hypertrophy

Strain pattern in corresponding leads

ECHO & Catheterization diagnostic

AS•1-50 age•Male

PS•5-15 aged

•MaleCOA

•1-5 aged•child

OBSTRUCTIVE

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ACYANOTIC OBSTRUCTIVE

Normal or slow peaking pulse

Low systolic normal diastolic BP


Heaving LV impulse Nil or minimal cardiomegaly

Systolic thrill and ESM in AA

Conduction of murmur to carotids Delayed and soft A2 ECG – LVH with

strain

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A valvular heart disease caused by the incomplete opening of the aortic valve

RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

AORTIC STENOSIS

Aortic valve obstruction pressure gradient across AV LV Hypertrophy

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ECG & CXR in Congenital AS

left ventricular hypertrophy

the typical left ventricular “strain”

AV ECHO in Congenital AS

a single closure line (arrow)

produced across the aorta

AV ECHO in Normal AV

Note the three cusps

"Mercedes Benz" configuration

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Angiogram in Congenital AS

Angiocardiogram from a 13-year-old boy

He has a discrete subaortic stenosis

2D ECHO Parasternal long-axis a 27-year-old man

a non-obstructive sub-aortic membrane (open arrow)

It was relatively remote from the aortic valve

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SV Aortic stenosis in Williams

Elfine Facies

a genetic disorder with autosomal dominant inheritance

2D ECHO in SV Aortic Stenosis

a single closure line (arrow) echogenic membrane is commonly observed above the sinuses of Valsalva

Aortogram in SV Aortic Stenosishourglass deformity diffuse hypoplasiadiameter of the ascending aorta is smaller than that of the aortic root

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Normal pulse and BP

Normal or elevated JVP

a>v

Left Parasternal Heave

Systolic thrill 2nd left space

Ejection systolic murmur ULSB

Wide but not fixed S2

Well heard but delayed P2

ECG RVH with strain

CXR – RV Cardiomegaly Pul oligemia

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A valvular heart disease in which outflow of blood from right ventricle is obstructed at the pulmonic valve

RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

PULMONARY STENOSIS

Pulmonary valve obstruction pressure gradient across PV

RV Hypertrophy

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Valvular Pulmonary Stenosis

Peak gradient across the valve is more than 50 mmHg

Peak gradient across the valve is less than 50 mmHg

CXR in Valvular Pulmonary Stenosis

RAE RVH and prominent pulmonary bay

Decreased pulmonary blood flow - oligemia

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Normal or high volume pulse

Radio-femoral delay

Arm BP > thigh BP

Prominent carotid and

palpable aorta

Palpable chest collaterals

ESM in the inter-scapular

area

Additional systolic and continuous

ECG LVH with strain

3 sign in the CXR

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RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

COARCTATION

Aortic Arch constriction pressure gradient across COA LV Hypertrophy

Bicuspid aortic valve

Obstruction to blood flow

Pre-ductal dilatation

Constriction of aorta

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Better developed upper extremities in COA

Most commonly found distal to the left subclavian artery, opposite the point of entry of the ductus arteriosus (juxtaductal). CoA can occur in other locations along the aorta

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Chest X-ray PARAA seen in up to 50% of patients

Cardiomegaly is present

Usually of left ventricular type

Rib notchingNotching of lower surface of ribs

Usually present bilaterally

Due to compression by collaterals

Barium Swallow "E" or "reversed 3" sign.

prestenotic dilatation of the aorta

Poststenotic dilatation of aorta

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Aortogram in COA

with contrast medium injected into the ascending aorta

Contrast injection into the descending aorta (Dao)

Echocardiogram in COA

A, 2D echo of aortic coarctation

TAA = transverse aortic arch

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ACYANOTIC L-R SHUNT

Sudden Chest pain

Bounding pulse

Wide pulse pressure



continuous thrill LSB

Superficial continuous

murmur

Murmur peaks in diastole

LV dominance in ECG

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RSOVAorta-RA Fistula in the same patient demonstrated at the time of surgery

Aortic Angiogram and echocardiography showing the leak from aortic root to RA

Non-coronary -RV

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ACYANOTIC L-R SHUNT

8 month old infant

Sudden chest pain

wide pulse pressure





murmur


Acute MI in ECG

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Coronary Arteriovenous FistulaCoronary arteriovenous fistula between LAD

and pulmonary arteryThe leash of vessels arising from the LAD is

draining into the pulmonary artery

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ACYANOTIC L-R SHUNT

Infant 6 months old

Chest pain unrecognised

unrelated to exertion

wide pulse pressure





murmur


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Anomalous origin of the left main coronary artery from the pulmonary artery

ALCAPA Natural History

A, In a fetus, both right and left coronary arteries receive

forward flow from their respective great arteries.

B, Soon after birth, before collaterals are well developed,

there may be an ALWMI and slight retrograde flow from

LCA to the pulmonary artery.

C, After collaterals have enlarged, there is high flow in

the enlarged RCA and the collaterals and significant

retrograde flow into the pulmonary artery

Typical electrocardiogram of an infant with

anomalous left coronary artery

before (above)

and after (below)

ligation of the anomalous left coronary artery. Note the abnormal Q waves in I, AVL, and V6.

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CYANOSIS AND CLUBBING

Failure in Devpt

Failure in mentation

Exertional Cyanosis

Squatting episodes

Clubbing

Polycythemia

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CYANOSIS AND CLUBBING

Normal pulse and BP


No cardiomegaly No thrill

Left Parasternal Heave

Second Heart sound single

ESM in Pulmonary Area

Murmur ends before A2

CXR MPA absent - Lung ischemic

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CYANOSIS & CLUBBING

PS with shunt

Ventricular level

RAD + RVH

Tetralogy of Fallot

RAD +LVH

Hypoplastic Right Ventricle

LAD + RVH

Critical Pulmonic Stenosis

LAD + LVH

Tricuspid Atresia

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RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

TETRALOGY OF FALLOT

Infundibular stenosis

Over-riding of aorta

Rt Ventricular Hypertrophy

Ventricular septal defect


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50

Trans-thoracic Echocardiogram in COA



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CYANOSIS & CLUBBING

PS with shunt

Atrial level

Normal pulse and BP

Systolic thrill

Prominent a in JVP

TR may be present

Cardiomegaly

RVH late transition

PULMONARY ATRESIA

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Pulonary Atresia

ostium secundum defect

endocardial

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Diagramatic Representation of Pulmonary Atresia



54

CYANOSIS & CLUBBING

PS with shuntAtrial level

As well as

Ventricular levelNormal pulse and BPSystolic thrill at LSB

Right atrial enlargement

Prominent a in JVPTR may be presentLeft Axis deviation

Cardio-megalyRVH late transition

Left Vent HypertrophyTRICUSPID ATRESIA

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Tricuspid Atresia

RA

RV

LA

LV

VC PV

PA AO

LUNG

SVC

IVC

TRICUSPID ATRESIA

Ventricular septal defect

Blood mixture in pul artery

Atrial septal defect

Tricuspid Atresia

Patients with concordant ventriculo-arterial connection tend to be mre cyanosed than those with discordant connection

56

CYANOSIS & CLUBBING

Relatively older patient

Features of PAH

Short Systolic Murmur

MPA Prominent

Peripheral pruning

57

enlarged central pulmonary arteries

peripheral pruning of pulmonary vessels

CXR in Eisenmenger syndrome due to VSD

58

peaked P wave in lead II

right ventricular hypertrophy

right-axis deviation.

ECG in Eisenmenger syndrome due to VSD

59

CYANOSIS & CLUBBING

PS with shunt-Atrial level

Quiet precordium

Gallop rhythm

Cardiomegaly

Scratchy systolic murmur

Multiple sounds

Scratchy diastolic murmur

EBSTEIN’S ANOMALY

61

ECG in Ebstein’s Anomaly

Short PR, delta wave& wide QRS

Peaked P wave in V2 - RAE

ECG in Ebstein’s Anomaly



62

2D ECHO 4C View

Septal attachment of TV

Massive RA dilatation

Necropsy specimen in EA



2D ECHO 4C View



63

CYANOSIS & CLUBBING

Neonate or infant

Failure to thrive

Cardiomegaly

Congestive Heart Failure

TRANSPOSITION OF GREAT VESSELS

64

Patent foramen ovale -

ostium secundum defect

endocardial cushion

65

CXR in CTGA

Vascular pedicle is narrow (paired white arrows)

Hump-shape caused by inverted infundibulum

ECG in CTGA

Atrial flutter at 200 beats/min

RAD, RVH and incidental VPC

66

CXR in CTGA

Vascular pedicle

Hump-shape

67

CYANOSIS & CLUBBING

InfantSevere Cyanosis

CV collapseIncreased Pul blood flowNormal Pul Artery PrLate onset PAHC/I for correctionECGRADRAERVH

Chest X-RayCardiomegalyPul PlethoraFigure of 8 heartTAPVCECHOCEMarkedly large RA RVSmall LACathNot neededMay be hazadousfor urgent septoplasty

68

PAPVC

Vascular pedicle

Hump-shape caused by inverted infundibulum

TAPVC

Atrial flutter at 200 beats/min

RAD, RVH and incidental VPC

69

enlarged central pulmonary arteries

peripheral pruning of the pulmonary vessels

TAPVC

70

CYANOSIS & CLUBBING

Infant

Increased blood flow

Normal PAP

Chest X-Ray

Without Cardiomegaly

No PAH

Peripheral pruning

Pulmonary AV Fistula

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SUMMARY

CHD

Acyanotic

LR shunt

ASD

VSD

PDA

Ao-Rh shunt

RSOV

CAVF

ALCAPA

Obstructive

AS

PS

COA

Cyanotic

With PS

TOF

PA

TA

PA With PAH

Eisenmenger ASD

Eisenmenger VSD

Eisenmenger PDA

With Normal or ↑PAP

EA

cTGA

PAVF

72

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cvs simple approach to chd

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