Congenital Heart Disease

1. Congenital Heart Disease

4. Cyanotic – Tetralogy of Fallot (TOF) • Most common form of CHD • 3-6 infants for every 10,000 births • TOF is sporadic and nonfamilial • Associated with 4 major heart defects • Ventricular septal defect (right to left shunt) • Narrowing of the pulmonary outflow tract • Overriding aorta • A thickened muscular wall of the right ventricle • Severity of TOF depends on degree of subpulmonarystenosis • Tx: surgery to enlarge pulmonary tract and close the VSD after birth or during infancy.

6. Transposition of Great Vessels (TGA) • 2nd most common CHD, 20-30 per 100,000 live births • Aorta and pulmonary veins positions are transposed • Results in separation of systemic and pulmonary circulation • Incompatible with post-natal life without shunts • Prognosis depends on... • degree of ‘mixing’ of blood • Stability of shunt • Ability of right ventricle to maintain systemic circulation • Without surgery, most die within months. • Reposition vessels, coronary bypass

8. Tricuspid Atresia • 1 per 10,000 live births • heart valve is missing or abnormally developed • Due to unequal division of AV canal during development • MV larger than normal, underdevelopment of RV • Circulation maintained by right-to-left shunt via ASD or patent foramen ovale and VSD • Definitive surgery is delayed to about 2 years of age • 3 surgeries: a) shunt aorta PA b) SVC  RPA c) SVC & IVC  PA

9. (Bidirectional cavo pulmonary connection)

11. Total Anomalous Pulmonary Venous Connection (TAPVC) • 6.8 per 100,000 live births • Condition where pulmonary veins fail to join the LA • Results: • Hypertrophy and dilation of right side of heart due to volume/pressure • Dilation of pulmonary trunk • LA hypoplastic • Patent foramen ovale or ASD is present allowing pulmonary venous blood to enter LA. • Surgery after birth or within 6 months of life. • PV  LA, closure of ASD

13. PersistantTruncasArteriosus • 5-15 of 100,000 live births • Failure of embryological division of aorta and pulmonary artery • Results in single great artery that receives blood from both ventricles, serving in systemic, pulmonary and coronary circulation • Presence of massive VSD • Too much blood goes to the lungs

15. Non Cyanotic CHD • Coarctation of the aorta • Aortic coarctation is a narrowing of part of the aorta. Aortic coarctation is more common in persons with certain genetic disorders, such as Turner Syndrome. • Pulmonary stenosis • Stenosis occurs when the valve cannot open wide enough. As a result, less blood flows to the lungs. There may also be narrowing just before or just after the valve. • Aortic stenosis • In aortic stenosis, the aortic valve does not open fully. As the aortic valve becomes more narrow, the pressure increases inside the left heart, causing the left heart ventricle to become thicker, which decreases blood flow and can lead to chest pain. Severe forms of aortic stenosis prevent enough blood from reaching the brain and rest of the body. This can cause light-headedness and fainting.

16. Left-to-Right Shunts • Atrialseptal defect (ASD) • Fixed opening between atria by incomplete tissue formation. Asymptomatic until adulthood • Patent foramen ovale • Small hole created by an open flap of tissue from septum. Normally closes at birth due to pressure on left side of heart. Closes in 80% of people. • Ventricular septal defect (VSD) • Incomplete closure of v-septum. Most common congenital cardiac anomaly, mostly associated with other congenital anomalies, only 20-30% isolated. • Patent ductusarteriosus (PDA) • Normally closes at birth. Fetal: shunts PA  Aorta to bypass lungs. 90% occur as isolated anomaly. • Atrioventricularseptal defect (AVSD) • Partial: ASD only, two valves, but one (usually mitral) is abnormal causing leaks between chambers of one side of heart. • Complete: All 4 chambers freely communicate, ASD + VSD. One large AV valve instead of mitral/tricuspid/