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Congenital heart disease Congenital cardiovascular defects congenital heart defects

2013 American Heart Association, Inc. All rights reserved. Unauthorized use prohibited.

Incidence
The most commonly reported incidence of congenital heart defects in the United States is between 4 and 10 per 1,000, clustering around 8 per 1,000 live births. Continental variations in birth prevalence have been reported, from 6.9 per 1000 births in Europe to 9.3 per 1000 in Asia.

Congenital Heart Defects

Commonly dibagi menjadi sianosis dan asianosis ACYANOTIC
LEFT RIGHT SHUNTS
Ventricular septal defect (30%) Patent ductus arteriosus (12%) Atrial septal defect (7%)

CYANOTIC
Tetralogy of Fallot (5%) Transposition of the great arteries (5%) Atrioventricular septal defect complete (2%)

OUTFLOW OBSTRUCTION
Pulmonary stenosis (7%) Aortic stenosis (5%) Coarctation of the aorta (5%)

Cyanotic Lesions
Ductal Dependent Increased pulmonary blood flow Decreased pulmonary blood flow Mixing Lesions

Cyanotic Lesions
Tetralogy of Fallot Transposition of Great Arteries Tricuspid Atresia Pulmonary stenosis

Cyanotic - TOF
Insiden 50% dari penyakit jantung sianotik Gambaran klasik dari 4 kelainan Large VSD Overriding aorta (dekstroposisi) Subpulmonary stenosis Right ventricular hypertrophy Klinis : dispneu, sianosis diperburuk saat menangis, bisisng sistolik , bunyi S2 lemah, 50% teraba getaran, Clubbing of fingers

Penunjang : rontgen toraks = jantung seperti sepatu boot ekg = RVH

 Investigations:

Cyanotic - TOF

boot shaped heart (upturned apex due to RVH) ECG RVH

Management
Initially medical Surgical at around 6 months Cyanosed neonates require a shunt Hypercyanotic spells > 15 mins:
Sedation and pain relief with morphine IV propanolol IV fluids Bicarbonate to correct acidosis Ventilation reduced metabolic demand

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)

Cyanotic - Transposition
Transposition of the great arteries Tertukarnya posisi aorta dan arteri pulmonaris, bersatunya atrium, bersatunya aorta dan arteri pulmonaris Klinis :
Cyanosis Muncul pada 2-3 hari setelah duktus tertutup Presentation delayed if there is VSD etc Muncul bisisng sistolik

Management: - memperbesar ASD Pembedahan

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)

Cyanotic - Tricuspid Atresia

Insiden : 2-5% dari semua CHD Kelainana : atresia trikuspid, ASD, hipoplasia ventrikel kanan dan VSD Klinis : hipoksia berat, tanpa perbaikan 90% meninggal dalam 1 tahun, sianosis, Diagnosis menggunakan ekokardiografi Terapi : prosedur fontan

ACYANOTIC
LEFT RIGHT SHUNTS
Ventricular septal defect (30%) Patent ductus arteriosus (12%) Atrial septal defect (7%)

OUTFLOW OBSTRUCTION
Pulmonary stenosis (10%) Aortic stenosis (5%) Coarctation of the aorta (10-15%)

Acyanotic - VSDs
Most common congenital heart defect, 2/1000 births Usually in the membranous part of the septum Classified by size:
Small asymptomatic which normally close spontaneously. May have a thrill, pansystolic murmur at the LLSE Large same/bigger than aortic valve. Present with breathlessness, HF, failure to thrive, difficulty feeding, recurrent chest infections after 1 week of age

 Patent ductus arteriosus when the ductus arteriosus does not close within 72 hours of birth (normally within 48 hours) Most common in preterm infants Normally kept open due to low fetal systemic arterial oxygen tension (PaO2) and elevated circulating prostaglandins (from placenta and lungs) At birth, PaO2 increases, and PG synthesis/supply falls contraction of oxygen and PG sensitive smooth muscle in the ductus arteriosus

Acyanotic - PDA

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)

Acyanotic Atrial Septal Defect

Two types: Secundum ASD (80%)
Defect in centre of atrial septum involving foramen ovale# Foramen ovale closes when baby takes first breath due to increased left atrial pressure

Partial atrioventricular septal defect

Associated with Downs syndrome

Clinical features
Commonly asymptomatic Recurrent chest infections/wheeze HF Arrhythmias in adulthood

 Pulmonary stenosis partial fusion of pulmonary valve leaflets Clinical features: Mostly asymptomatic Ejection systolic murmur at upper left sternal edge Soft/absent S2 Prolonged RV impulse with delayed valve closure in severe cases

Acyanotic - PS

Investigations:
ECG RVH

Management
Transcatheter balloon dilatation

 Aortic stenosis partial fusion of aortic valve leaflets (1-3 leaflets) Clinical features: Asymptomatic Chest pain, syncope, reduced exercise tolerance if severe Low volume, slow rising pulses Carotid thrill Ejection systolic murmur at R sternal edge neck

Acyanotic - AS

Investigations:
ECG LVH post-stenotic dilation of aorta may be seen

Management
Transcatheter balloon valvotomy when presssue gradient >64 mmHg

 Coarctation of the aorta constriction in the diameter of the aorta, most usually after the site of the ductus arteriosus Clinical features: Can present as neonatal collapse HF Weak/absent femoral pulses In adults: raised BP (particularly R arm), radiofemoral delay, ejection systolic murmur Management: Stenting Surgical repair

Acyanotic - Coarctation

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)