CHD pdf - D. Tahsin - Muhadharaty

Congenital Heart Disease

Scope

• Fetal circulation Vs mature circulation
• Development of pulmonary HT in CHD
• CHD with shunts: ASD, VSD, PDA
• CHD without shunts: congenital PS,

Co-A

• Cyanotic CHD: TOF

Objectives

• CHD can manifest for the first time in adulthood
• CHDs with shunt have similar clinical presentations
• PHT & Eisenmenger’s syndrome may complicate all

conditions with increased pulmonary blood flow

(including shunt lesions) if untreated

Objectives

• Initially, PHT develops due to increased flow & is

usually reversible; later due to increased pulmonary

vascular resistance & is irreversible

• Recognition depends on the underlying anatomical

defect and its hemodynamic consequences

The Normal Circulation

Congenital Heart Disease:

Classification

• Acyanotic:

– With shunt: e.g. ASD, VSD, PDA
– Without shunt: e.g. PS, coarctation of

the aorta, congenital AS, congenital

MS……

• Cyanotic

– With reduced pulmonary blood flow: e.g.

TOF

– With increased blood flow: TGA

Common Congenital Heart Disease

• Atrial septal defect (ASD)

– Osteum secundum
– Osteum primum

• Ventricular septal defect (VSD)
• Patent ductus arteriosus (PDA)
• Coarctation of the aorta
• Congenital pulmonary stenosis
• Tetralogy of Fallot (TOF)

Clinical Presentation of CHD

• Asymptomatic
• Congestive heart failure
• Cyanosis and digital clubbing
• Failure to thrive
• Recurrent chest infections

Clinical Presentation of CHD

• Heart murmur

• Pulmonary hypertension with reversed shunt

(Eisenmenger syndrome)

Pulmonary Hypertension

• Initially caused by increased blood flow through the

pulmonary vessels due to left-to-right shunt

• Usually reversible on correction of the defect

Pulmonary Hypertension

• Later on: structural changes affect the walls of

pulmonary arterioles, including:

– Arterial wall thickening
– intraluminal thrombosis
– Capillary obliteration

• Probably irreversible!

Pulmonary Hypertension

These structural changes leads to:

• increased resistance to pulmonary blood flow
• Reduction of pulmonary blood flow
• Right-to-left shunt through the connection between

the two circulations (reversed shunt, the

Eisenmenger’s syndrome)

Eisenmenger

’s Syndrome:

clinical features

• Cyanosis and clubbing
• Raised JVP
• Left parasternal heave (RVH)
• Systolic expansion of the pulmonary

artery

• Palpable second heart sound
• Loud pulmonary second sound

Eisenmenger

’s Syndrome:

clinical features

• RV third heart sound
• Murmurs:

– early diastolic murmur at the pulmonary

area (Graham-Steel murmur)

– Tricuspid regurgitation (pansystolic

murmur at LSB)

Eisenmenger

’s Syndrome: ECG

• Right axis deviation

• Right ventricular hypertrophy (tall R

waves in V1& V2)

• Peaked P wave (RA enlargement)

ECG in Eisenmenger

’s Synd.

ECG in Eisenmenger

’s Synd.

CXR in Eisenmenger

’s Synd.

• CXR shows enlarged central pulmonary arteries &

peripheral pruning of the pulmonary arteries

CXR in Eisenmenger

’s Synd.

Complications of

Cyanotic

Heart

Disease

• Polycythemia: hyperviscosity syndrome
• Hemoptysis, sometimes massive and

fatal

• Paradoxical embolization
• Brain abscess

Specific Congenital Heart

Diseases

Atrial Septal Defect (ASD)

• Osteum primum ASD:

– part of endocardial cushion defects
– associated with mitral regurgitation and tricuspid

regurgitation

• Osteum secundum ASD: at the area of fossa ovalis

ASD: Pathophysiology

• Shunting of blood

from LA to RA

through the defect

leads to dilatation

of RA, RV, & PA,

but not LA or LV

ASD: Symptoms

• Dyspnea
• Recurrent chest infections
• Heart failure
• Arrhythmias (palpitations)

ASD: Signs

• ↑ JVP
• Left parasternal heave
• Fixed splitting of S2
• Systolic murmur at the pulmonary area
• NO THRILL is felt at the pulmonary area (unlike

valvular PS)

ASD: Investigations

• ECG:
• CXR
• Echocardiography
• Trans-esophageal echocardiography (TEE)

ASD: ECG

• Incomplete RBBB

• With secundum ASD: right axis deviation

• With primum ASD: left axis deviation

Ostium Secundum ASD

Ostium Primum ASD

ASD: CXR

• Dilated RV, RA, and PA

• plethoric lungs: increased pulmonary arterial and

venous markings

ASD: CXR

Echocardiography & TEE

• Shows the size of the defect

• The direction of blood flow

• The pulmonary artery pressure

Ostium Secundum ASD

Ostium Primum ASD

ASD: Management

• Surgical closure when the shunt is large (exceeds

1.5:1)

• Recently: closure with implantable closure devices

during cardiac catheterization

• Endocarditis prophylaxis for primum ASD

ASD: Management

• Endocarditis prophylaxis is not required in osteum

secundum ASD unless associated with other valvular

or congenital defects

Ventricular Septal Defect

(VSD)

Ventricular Septal Defect (VSD)

• Failure of septation

of the ventricles

• The interventricular

septum is normally

composed of small

membranous and

large muscular parts.

Ventricular Septal Defect (VSD)

• The usual position of

the defect is around

the membranous

septum

(perimembranous

VSD)

VSD: Pathophysiology

• The magnitude of the

shunt depends on the

size of the defect &

the relative systemic &

pulmonary resistance

VSD: Pathophysiology

• The shunt involves the

LV, RV, PA, PVs, & LA

VSD: Pathophysiology

• The shunt does not

involve the RA or

the aorta

• There is increased

flow through the

mitral valve & LV

volume overload

VSD: Clinical Presentation

• Dyspnea
• Recurrent chest infections
• Heart failure
• Accidental finding of a murmur
• Eisenmenger’s syndrome

VSD: Physical Findings

• Hyperdynamic apex beat
• Systolic thrill: flow through the

defect

• Physiological splitting of S2 (↑ with

breathing)

• S3: LV volume overload

VSD: Physical Findings

• LV-RV shunt causes pansystolic murmur at the left

sternal border

• Increased flow through the mitral valve causes

diastolic murmur at the apex

VSD: Investigations

• ECG
• CXR
• Echocardiography

VSD: CXR

• Plethoric lungs
• Prominent main pulmonary artery
• LA dilatation
• Cardiomegaly of LV configuration

CXR of VSD

VSD: ECG

• LVH: Tall R waves in V5 & V6 & Deep S waves in

V1 & V2

• Biventricular hypertrophy: tall R in V1 & V2, tall R

in V5 & V6

ECG in VSD: Biventricular Hypertrophy

VSD: Echocardiography

VSD: Treatment

• Small defects:

– no indication for surgical closure
– Attention should be paid for endocarditis prophylaxis

VSD: Treatment

• Large defects with heart failure:

– Medical treatment: digoxin, diuretics, ACEIs
– Definitive treatment: surgical repair of the defect
– Lately: closure by catheterization (occluder)

VSD: Treatment

• If the Eisenmenger’s syndrome has developed:

– Heart-lung transplantation