CHD pptx - 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
2

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
4

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

7

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)
8

Clinical Presentation of CHD

Asymptomatic
Congestive heart failure
Cyanosis and digital clubbing
Failure to thrive
Recurrent chest infections
9

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

11

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
14

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)
16

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
21

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
23

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)
26

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)

27

ASD: Investigations

ECG:
CXR
Echocardiography
Trans-esophageal echocardiography (TEE)

28

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
32

ASD: CXR

Echocardiography & TEE

Shows the size of the defect

The direction of blood flow

The pulmonary artery pressure
34

Ostium Secundum ASD

Ostium Secundum ASD

Ostium Primum 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
42

ASD: Management

Endocarditis prophylaxis is not required in osteum secundum ASD unless associated with other valvular or congenital defects

43

Transcatheter Closure of ASD

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

52

VSD: Physical Findings

Hyperdynamic apex beat
Systolic thrill: flow through the defect
Physiological splitting of S2 (↑ with breathing)
S3: LV volume overload
53

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
55

VSD: CXR

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

56

CXR of VSD
57

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

64

VSD: Treatment

Large defects with heart failure:
Medical treatment: digoxin, diuretics, ACEIs
Definitive treatment: surgical repair of the defect
Lately: closure by catheterization (occluder)
65

VSD: Treatment

If the Eisenmenger’s syndrome has developed:
Heart-lung transplantation
66