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also known as ..

• 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

asianosisACYANOTIC

LEFT RIGHT SHUNTSVentricular septal defect (30%)Patent ductus arteriosus (12%)Atrial septal defect (7%)

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

CYANOTIC

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

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

Cyanotic - TOF• Investigations:

– 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

USSShowing 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

USSShowing 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

Acyanotic - PDA• 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

USSShowing 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 Down’s syndrome

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

Acyanotic - PS• 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

• Investigations:– ECG – RVH

• Management– Transcatheter balloon dilatation

Acyanotic - AS• 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

• Investigations:– ECG – LVH

– post-stenotic dilation of aorta may be seen

• Management– Transcatheter balloon valvotomy when presssue gradient >64

mmHg

Acyanotic - Coarctation• 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), radio-

femoral delay, ejection systolic murmur• Management:

– Stenting– Surgical repair

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