Approach to a patient with congenital heart disease

Guide : Dr.Vijay G.Somannavar

Introduction

• Congenital heart diseases or Congenital heart anomalies are defects in the structure of the heart and great vessels which are present at birth.

•  CHD are the leading cause of birth defect-related deaths.

D E F I N I T I O N

When to suspect CHD

•-Cyanosis -Respiratory distress -Poor peripheral

perfusion -Decreased pulsations-Difference in pulses

(arm vs. leg)

-Single 2nd heart sound -Abnormally loud 2nd

heart sound -Prominent heart

murmur -Hyperactive precordium

F I N D I N G S T H A T S H O U L D A L E R T O N E T O T H E P O S S I B I L I T Y O F C H D

Incidence

• 1 in 10 stillborn infants have a cardiac anomalies.

• 8 out of 1000 live born children have significant cardiac malformations.

• Every year 1,80,000 children are born with heart defects in India.

Lesions % of all Lesions

- Ventricular septal defect 25-30

- Atrial septal defect (Secundum) 10

- Patent ductus arteriosus 10

- Coarctation of aorta 7

- Tetralogy of Fallot 6

- Pulmonary Valve Stenosis 5-7

- Aortic Valve Stenosis 4-7

- Transposition of great arteries 4

- 0thers 20

Classification

CHD

Acyanotic Cyanotic

Left-to-right shunts

Outflow obstruction

- Ventricular Septal Defect (VSD)- Patent Ductus Arteriosus (PDA)- Atrial Septal Defect (ASD

- Pulmonary Stenosis- Aortic Stenosis-Coarctation of aorta

• Teralogy of fallot• Tricuspid atresia• Transposition of

great vessels• Truncus arteriosus• Total anomalous

pulmonary venous return (TAPVR)

• Ebstein’s anomaly

Etiology

Maternal disorders

• Rubella infection (30-35%)• PDA,

pulmonary stenosis

• SLE (35%)• Complete heart

block• DM (2%)

Maternal drugs

• Warfarin therapy (5%)• PDA,

pulmonary stenosis

• Fetal alcohol syndrome (25%)• ASD, VSD,

tetralogy of Fallot

Chromosomal abnormality

• Down’s syndrome (30%)• Atrioventricular

septal defect, VSD

• Edward’s and Patau’s syndrome (60-80%)• Complex

• Turner’s syndrome (15%)• Aortic valve

stenosis, coarctation of the aorta

Left to right shunts

• L to R shunts are characterised by RV enlargement and RV failure.

• Usually present with recurrent chest infections.

• They are not typically cyanotic.

Ventricular Septal Defect (VSD)

• Most common congenital cardiac lesion.• Accounts for 25% of all CHDs. • Defect may be –

perimembranous(adjacent to tricuspid valve) or muscular(surrounded by muscle)

• Spontaneous closure occurs by 10 years.

Peri membranous

Muscular

Clinical features

• Children with small defects will remain asymptomatic .

• Infants with moderate to large defects will become symptomatic within the first few weeks of life.

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S I G N S A N D S Y M P T O M S V A R Y W I T H T H E S I Z E O F T H E D E F E C T

Small VSDs

– Symptoms• Asymptomatic

– Physical signs• Thrills at lower sternal edge• Loud pansystolic murmur at lower left sternal edge• Quiet second heart sound (P2)

Large VSDs

– Symptoms• Breathlessness and failure to thrive.• Recurrent chest infections

– Physical signs• Prominence of the left precordium• Soft pansystolic murmur • Mid-diastolic murmur at the apex• Loud pulmonary second sound (P2)

Investigations

– Chest X-ray • Cardiomegaly• Enlarged pulmonary arteries• Pulmonary vascular markings• Pulmonary oedema

– ECG• Biventricular hypertrophy and

signs of pulmonary HTN right ventricular enlargement and hypertrophy

– Echocardiography• Demonstrates the anatomical

defect, haemodynamic effects and severity of pulmonary HTN.

Atrial Septal Defect(ASD)• Seen in 10% of all CHDs

• Females > males.

• It is an abnormal opening between the atria

• Spontaneous closure ocurrs with in 1st year of life.

Clinical features

Symptoms :• Fatigue and SOB• Palpitations • Recurrent respiratory

infections

Physical signs :• Ejection Systolic murmur • Diastolic murmur (large

shunts).• Wide fixed split S2• Tachypnea, tachycardia and

enlarged liver from heart failure

M O S T I N F A N T S A N D C H I L D R E N A R E A S Y M P T O M A T I C .

Diagnostic tests

- CXR – enlarged heart,increased pulmonary vascular markings

- ECG- Ostium secundum:Right axis deviation with Right bundle branch block.

- 2D echo – show pattern of blood flow through the septal opening

Patent Ductus Arteriousus (PDA)

• PDA occurs in 6-11 % of all children with CHD

• It is a connection between the aorta and the pulmonary artery

• Most babies have a closed ductus arteriosus by 72 hours after birth.

Clinical features

• Fatigue • Sweating • Tachypnea • Shortness of breath

Physical examination• Widened pulse pressure

• Collapsing/ bounding pulse

• Left infraclvicular/upper left sternal edge continuos machinery

murmur

• Differential cyanosis (cyanosis of lower limb but upper limb pink)

Coarctation of aorta

• Accounts for 7 % of all CHD.• is narrowing of the aorta at varying

points anywhere from the transverse arch to the iliac bifurcation.

• 98% of coarctations are juxtaductal.

Clinical presentation

• In older children:– Leg discomfort with

exercise– Headache– Epistaxis

• Systolic hypertension of upper extremities.

• Ejection systolic murmur at upper sternal edge

• Diminished lower extremity pulses– Radio-femoral delay:

• blood bypassing the obstruction via collateral vessels in the chest wall

Right to Left Shunts

• R L shunts cause hypoxia and central cyanosis.

• Blood is shunted from the R to the L side of the heart w/o passing through the lungs for oxygenation.

• Unoxygenated blood circulates in arteries cyanosis

Tetrology of fallot

Components• Ventricular septal defect• Pulmonary stenosis• Overriding of aorta• RVH

M O S T C O M M O N C Y A N O T I C H E A R T D I S E A S E

Clinical features

– Cyanosis– Shortness of breath– Rapid breathing– Loss of consciousness– Clubbing of fingers and toes– Restless and agitated– Poor weight gain

S Y M P T O M A T I C A N Y T I M E A F T E R B I R T H

Posture

– It is a compensatory mechanism

– Squatting increases the peripheral vascular resistance, diminishes the right-to-left shunt and increases pulmonaryblood flow.

S I T T I N G P O S T U R E O R S Q U A T T I N G

• Chest X-ray– Normal sized, boot

shaped heart– Concavity of Left heart

border– Oligaemic lung fields

• ECG– At birth normal– Older: Right axis

deviation and RVH

• Echocardiography– Degree of stenosis– Coronary anomalies(5%

in TOF)

Thank You