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CONGENITAL HEART�DISEASES

Dr. SALEEM HASAN

MUKKADDAM

CONGENITAL HEART DISEASE

Definition: Born with Heart disease.

Incidence: 8/1000 Live birth.

80/1000 still birth.

VSD: 32%

PS:

ASD:

COA: PDA

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6% - 8%

• AS
• MVP
• TOF
• TGA
• 3 shunts (VSD, ASD, PDA)
• 3 outflow obstruction (AS, COA, PS)
• 2 transpositions (TOF, TGA): 70 – 80% of all congenital heart diseases

3% - 8%

3% - 5%

Classification of congenital heart diseases

1. Acyanotic

1. Shunt anomaly

Ventricular septal defect (VSD)

Atrial septal defect (ASD)

Persistent ductus arteriosus (PDA)

11. Lesions at, below or above a valve

Aortic valve:

Bicuspid aortic valve

Aortic atresia

Subaortic stenosis and Hypertrophic

obstructive cardiornyopathy

Supravalvular aortic stenosis

Coarctation of aorta.

Pulmonary valve:

• Pulmonary stenosis
• Subvalvular pulmonary stenosis and

pulmonary atresia.

• Pulmonary artery stenosis
• Idiopathic dilatation of pulmonary artery and pulmonary artery aneurysm.

Tricuspid valve: Ebstein's anomaly

Mitral valve: Juvenile Mitral stenosis Parachutte mitral valve

Miscellaneous:

Anomalous coronary artery

Dextrocardia

Congenital complete Heart Block.

(B) Cyanotic

1. Tetralogy of Fallot (TOF)

2. Transposition of great vessels (TGA)

5T. 3.Tricuspid atresia (TA)

4.Total anomalous pulmonary venous drainage. (TAPVD)

5. Persistence Trunchus (PTA)

6. Aortic atresia (AT) 2AT

7. Pulmonary atresia (PAT)

Causes of congenital heart diseases

A. Genetic

1. Chromosomal abnormality

Down's syndrome (Trisomy 21)

Small Head

Shallow orbit

Epicanthic fold

Protruding tongue

Transverse palmer crease (Simian crease)

Mental retardation

Fond of music

Associated congenital heart disease

AV canal defect

VSD

Others‑ ASD, PDA, TOF.

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Trisomy 13,18:

Association : VSD

Bicuspid aortic valve

Turner's syndrome (XO)

Short stature

Web neck broad chest with wide apart nipple

Primary amenorrhoea Common association

Coarctation of aorta, Pulmonary stenosis

Klinefelter's syndrome (XXY)

Tall stature

Enuchoid appearance

Gynaecomastia, small testicle

Common association‑ ASD

II. Mandellian inheritance

Autosomal dominant

ASD 2'

Coarctation of aorta

Congenital heart block

HCM

Marfan's syndrome

WP

PPH

Supravalvular AS

Autosomal recessive

Ebstein's anomaly

ASD

Homocysteinuria

X‑linked

Dextrocardia with other cardiac malformations

III. Multifactorial (poly2enic)

B. Environmental :

Rubella & other viruses

Drugs‑Lithium, phenytoin, Steroid, ACE inhibitors

Immunosupressive drugs, Alcohol,

Radiation

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ATRIAL SEPTAL DEFECT

Definition :

Abnormal communication in interatrial septum, which permit flow of oxygenated blood in the right atrium

Incidence

Common congenital heart disease in adult.

Next to MVP and Bicuspid aortic valve.

Clinical types :

Ostium. seccundum. At the level of foramen ovale Commonest variety ‑ 70% Ostium. primum ‑ 20% The defect is close to AV valves‑ partial or complete atrioventricular septal defect (endocardial cushion detect). Sinus venosus type‑ 5% One or two pulmonary veins attached to SVC or upper right atrium.

Clinical features:

Asymptomatic Incidental diagnosis Age: Late teen or 20 ‑ 30 years, even may be unnoticed in the elderly. First presentation may be during pregnancy.

Symptoms :

Palpitation (Volume overload, Arrhythmia)

Shortness of breath

Fatigue

CUF ‑ Early feature in ASD primum

Signs:

Wide fixed Splitting Of P2 is the diagnostic hallmark of ASD

Investigations:

ECG: Incomplete RBBB

RVH‑ late feature

CXR: Heart size may be normal or dilated with shifting of the Heart to the left. PA, dilated, plethoric lung field.

ECHO:

Confirm the diagnosis and site of origin

Evaluation for surgery.

Complications

1 . CHF

2. Atrial arrhythmias

3. Eissenmenaer syndrome

4. Paradoxical embolism or in situ pulmonary artery thrombo­embolism.

Differential diagnosis:

1Mitral stenosis

Mild pulmonary stenosis

Cor pulmonale (in later age).

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Treatment :

Surgical correction.

VENTRICULAR SEPTAL DEFECT

• Commonest congenital heart disease in childhood. One third of all congenital heart diseases.

Clinical type:

1 .Paramembranous (75%)

2. Muscular (15%)

3. Supracristal (Subaortic or subpulmonic : 8%)

4. Inlet or AV canal type : 2‑3%

Clinical features:

Depends on site and size of lesion.

Small defect:

No Symptoms

Loud tearing pansystolic murmur in LSE.

Infective endocrditis

Large defect:

Repeated respiratory infection in childhood

Always look for VSD

Breathlessness of exertion

CHF: (Biventricular failure)

Fatigue

Eissenmenger syndrome

ECG Normal ‑ LV high voltage ECG

Late ‑ Biventricular Hypertrophy

CXR: Cardiomegaly

Note: Cardiomegaly in childhood, think of VSD unless proved otherwise.

Echo with doppler :

• Confirm diagnosis
• Site and severity of lesion
• Associated lesion
• Assessment for surgery

Treatment

• Small VSD: More than one third spontaneously closed by 10 years
• Larger VSD: Surgical correction.

PERSISTENT DUCTUS ARTERIOSUS (PDA)

Definition

Extracardiac shunt, represent persistent patency of the vessel that normally connects pulmonary arterial system and aorta in the fetus.

Causes:

I .Exposure to rubella virus in 1^st. trimester of pregnancy.

2. Prematurity (birth wt < 1.5 kg) particularly with birth asphyxia

3. Birth in high altitude

Physiology

Functional closure within few hours due to 🡱0₂ tension & PGE2

Anatomical closure by fibrosis: 2 wks. ‑ 2 months.

(Ligamentum. arteriosum)

Clinical features:

1. F>M

2. Asymptomatic

3. Palpitation

4. CCF in infancy

5. Infective endocarditis

Signs:

Continuous machinery murmur in the left upper sternal border and below left clavicle is characteristic.

Diagnosis

ECG

CXR‑ normal‑ Cardiomegaly LV type apex, plethoric lung field.

ECHO with doppler : For Anatomical diagnosis & functional assessment.

Management: Medical

Surgical correction.

TETRALOGY OF FALLOT (TOF)

Commonest cyanotic congenital heart disease in

childhood.

Components:

Primary components:

1 . Pulmonary stenosis (Subvalvular ± valvular)

2. Large VSD

Secondary components:

3. Dextroposition of aorta with 30% to 40% overriding to theVSD.

4. Right Ventricular Hypertrophy

Clinical features:

Growth retardation

Central cyanosis

Clubbing

Cyanotic spell (Hypoxic spell)

Improves after squatting position

Single & Loud A2, ESM (PS), Continuous murmur (Around the scapula due to collaterals)

Polycythemia and its consequences

Gout

Acne

Gingivitis

Cerebral thrombosis

ECG:

RVH with extreme right axis deviation.

CXR:

Heart shadow ‑ Typically boot shaped with concavity of pulmonary segment (coeur in sabot), oligaemic lung field.

ECHO & Cardiac Catheterisation:

For confirmation of diagnosis & plan for surgery.

Management

Medical:

Management of cyanotic spell ‑ Squatting position

High concentration oxygenation

Morphine 0. 1 mg./kg body wt.

Surgical:

Palliative Blalock Taussig shunt (BT shunt)

Total correction.

Coarctation of aorta

Def: Narrowing of the lumen usually just beyond the origin of left subclavian artery.

Associations:

• Bicuspid aortic valve (50%)
• PDA, VSD
• Turner’s symdrome

Clinical features:

• May produce no symptom
• Incidental diagnosis

Diagnostic clue:

• Radiofemoral delay
• Upper body hypertension
• Lower limbs poorly developed
• Collaterals may be seen & felt around the scapula.

Investigations:

ECG: Normal

LVH in long standing cases

CXR: Aortic nuckle may be high or low figure of 3 may be seen in barium swallow esophagus

Rib notching of under side of ribs due to errosion by enlarged intercostal arteris. Upper 2 ribs are spared as they are supplied by costocervical branch of subclavian artery. 3^rd to 9 pairs are supplied by posterior intercostal artery branch of thoracic aorta.

Diagnosis:

Clinical grounds.

Confirm by:

Arch aortography & Intraaortic pressure tracing above & below the coarctation.

Management:

• Surgical resection of coarctation with end to end anastomosis.
• Balloon angioplasty: Particularly effective who have recurrence of coarctation after surgery.