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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
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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


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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
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The Normal Circulation

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CHD


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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

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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)
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Clinical Presentation of CHD

Asymptomatic
Congestive heart failure
Cyanosis and digital clubbing
Failure to thrive
Recurrent chest infections
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Clinical Presentation of CHD

Heart murmur

Pulmonary hypertension with reversed shunt (Eisenmenger syndrome)

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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

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Pulmonary Hypertension

Later on: structural changes affect the walls of pulmonary arterioles, including:
Arterial wall thickening
intraluminal thrombosis
Capillary obliteration

Probably irreversible!

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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)


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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
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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)


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Eisenmenger’s Syndrome: ECG

Right axis deviation

Right ventricular hypertrophy (tall R waves in V1& V2)

Peaked P wave (RA enlargement)
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ECG in Eisenmenger’s Synd.

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ECG in Eisenmenger’s Synd.

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CXR in Eisenmenger’s Synd.

CXR shows enlarged central pulmonary arteries & peripheral pruning of the pulmonary arteries


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CXR in Eisenmenger’s Synd.

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Complications of Cyanotic Heart Disease

Polycythemia: hyperviscosity syndrome
Hemoptysis, sometimes massive and fatal
Paradoxical embolization
Brain abscess
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Specific Congenital Heart Diseases

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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
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ASD: Pathophysiology

Shunting of blood from LA to RA through the defect leads to dilatation of RA, RV, & PA, but not LA or LV


CHD


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ASD: Symptoms

Dyspnea
Recurrent chest infections
Heart failure
Arrhythmias (palpitations)
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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)

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ASD: Investigations

ECG:
CXR
Echocardiography
Trans-esophageal echocardiography (TEE)

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ASD: ECG

Incomplete RBBB


With secundum ASD: right axis deviation

With primum ASD: left axis deviation


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Ostium Secundum ASD

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Ostium Primum ASD

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ASD: CXR

Dilated RV, RA, and PA

plethoric lungs: increased pulmonary arterial and venous markings
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ASD: CXR

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Echocardiography & TEE

Shows the size of the defect

The direction of blood flow

The pulmonary artery pressure
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Ostium Secundum ASD

CHD


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Ostium Secundum ASD

CHD


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Ostium Primum ASD
CHD


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Ostium Primum ASD

CHD


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CHD


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CHD


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CHD


CHD


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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
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ASD: Management

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

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Transcatheter Closure of ASD

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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.


CHD


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Ventricular Septal Defect (VSD)

The usual position of the defect is around the membranous septum (perimembranous VSD)

CHD


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VSD: Pathophysiology

The magnitude of the shunt depends on the size of the defect & the relative systemic & pulmonary resistance
CHD


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VSD: Pathophysiology

The shunt involves the LV, RV, PA, PVs, & LA

CHD


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VSD: Pathophysiology

The shunt does not involve the RA or the aorta
There is increased flow through the mitral valve & LV volume overload


CHD


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VSD: Clinical Presentation

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

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VSD: Physical Findings

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

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VSD: Investigations

ECG
CXR
Echocardiography
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VSD: CXR

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

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CXR of VSD
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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

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ECG in VSD: Biventricular Hypertrophy

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VSD: Echocardiography

CHD


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CHD


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CHD


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CHD


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VSD: Treatment

Small defects:
no indication for surgical closure
Attention should be paid for endocarditis prophylaxis

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VSD: Treatment

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

If the Eisenmenger’s syndrome has developed:
Heart-lung transplantation
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رفعت المحاضرة من قبل: Mubark Wilkins
المشاهدات: لقد قام 13 عضواً و 333 زائراً بقراءة هذه المحاضرة








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