1. Congenital Heart Disease Most occur during weeks 3 to 8 Incidence 6 to 8 per 1,000 live born births Some genetic – Trisomies 13, 15, 18, & 21 and Turner syndrome Environmental – congenital rubella Most cases the cause is not apparent

2. Congenital Heart Disease Malformation causing a left to right shunt Malformation causing a right to left shunt Malformation causing an obstruction

3. Shunts Right to left – cyanosis Tetralogy of Fallot Transposition Persistent truncus arteriosus Tricuspid atresia Clubbing and polycythmia

4. Left to Right Shunts Cause increase pulmonary blood flow – no cyanosis early on Leads to pulmonary hypertension Right ventricular hypertrophy As pulmonary vessels respond – raising pressure Leads to increase right ventricular pressure Right to left flow and cyanosis – late (tartive)

5. Atrial Septal Defect Not patent foramen ovale – 25% of normal 3 types: Secundum - most common-90% Primum – associated with cleft mitral valve Sinus venosus – anomalous connections of right pulmonary veins

6. ASD Left to right shunt Pulmonary resistance less Compliance of right ventricle is greater than the left Pulmonary blood flow 2-4 times normal Well tolerated until 30’s Less than 10% develop pulmonary hypertension

7. ASD

9. Ventricular Septal Defect Incomplete closure of the ventricular septum Roger’s disease Most associated with other defects (tetralogy) 30% are isolated Many spontaneous close over time 90% in the membranous septum

10. VSD Most the size of aortic orifice Left to right Right ventricular hypertrophy and pulmonary hypertension Leads to shunt reversal, cyanosis and death

11. VSD

13. Patent Ductus Arteriosus 90% isolated Continuous harsh murmur Left to right Obstructive pulmonary vascular disease gradually reverses shunt Close as soon as feasible May need to keep open if other abnormalities exist

14. PDA

16. Atrioventricular Septal Defect Partial – 1 0 ASD and cleft anterior mitral leaflet Complete – common AV valve 1/3 have Down’s syndrome

17. Right-to-Left Shunts – Early Cyanosis “T” – tetralogy, transposition of great arteries, tricuspid atresia, total anomalous pulmonary venous connections, and truncus arteriosus

18. Tetralogy of Fallot VSD Obstruction to the right ventricular outflow tract (subpulmonary stenosus) Aorta that overides the VSD Right ventricular hypertrophy Anterosuperior displacement of their infundibular septum

19. Tetrology

20. Tetrology of Fallot

21. Transposition of Great Vessels Abnormal formation of the truncal and aortopulmonary septa Aorta arises from the right ventricle and lies anterior and to the right of the pulmonary artery USD in about 35% - stable Patent formen ovale or PDA – unstable shunt Right ventricular hypertrophy

22. Transposition

24. Truncus Arteriosus Failure of separation of the embryologic truncus arteriosus into the aorta and pulmonary artery

25. Tricuspid Atresia Unequal division of the AV canal Underdevelopment of right ventricle ADS or VSD

26. Total Anomalous Pulmonary Venous Connection No pulmonary veins directly joins the lt atrium Results from when the common pulmonary vein fails to develop Primative systemic vein persist to drain lungs ASD or patent foramen ovale

27. Coarctation of Aorta 2:1 males Infantile – hypoplasia of aorta – proximal to PDA Adult form – distal to Ductus 50% accompanied by a bicuspid aortic valve

29. Pulmonary Stenosis and Atresia

30. Pulmonary valve stenosis

31. Aortic Stenosis and Atresia Valvular Hypoplastic, dysplastic, abnormal in number Subvalvular supravalvular