The document summarizes various congenital heart defects that can cause cyanosis in infants, including tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, total anomalous pulmonary venous return, tricuspid atresia, pulmonary atresia, and Ebstein's anomaly. It describes the characteristic features, causes, evaluations, and treatments for each condition. For the scenario presented, the assistant would start prostaglandin E1 treatment and call cardiology to perform an echocardiogram to determine the specific heart defect.

1. Congenital Heart Disease: Cyanotic Lesions Martin Tristani-Firouzi December 2005

7. Chronic cyanosis causes clubbing of the digits

9. The basis of congenital heart disease is rooted in an arrest of or deviation in normal cardiac development

13. T of F occurs as a result of abnormal cardiac septation Normal development Abnormal development

18. Surgical repair: Tof F

20. d-TGA results from abnormal formation of aortico-pulmonary septum http://www.med.unc.edu/embryo_images/ PA AO cushion LV RV LA RA truncus

25. Balloon atrial septostomy (Rashkind procedure)

26. Surgical management of d-TGA: Arterial switch procedure The arterial trunks are transected and “switched” to restore “normal” anatomy The coronary arteries are resected and re-implanted.

28. Truncus Arteriosus RV LV RAA LAA PA Ao Tr

29. Truncus arteriosus: aortico-pulmonary septum fails to develop http://www.med.unc.edu/embryo_images/ PA AO cushion LV RV LA RA truncus

30. Conotruncal development is dependent upon normal migration of neural crest cells Neural crest tissue is required for formation of: conotruncus aortic arches facial structures thymus parathyroid

32. Surgical correction: Truncus Arteriosus

33. Surgical correction: Truncus Arteriosus

35. Embryological basis of TAPVR Systemic venous connections reabsorb as PV confluence fuses w/ left atrium PV confluence PV confluence fails to fuse w/ left atrium Systemic venous connections persist

36. Venous connections in TAPVR Supracardiac: Ascending vertical vein most common Cardiac: RA or coronary sinus Infracardiac: Descending vein to portal system supracardiac cardiac infracardiac

38. Supracardiac TAPVR Pulmonary vein ascending vertical vein superior vena cava ascending aorta

39. TAPVC - Supracardiac

40. Supracardiac TAPVC - CXR “ Snowman ” appearance secondary to dilated vertical vein, innominate vein and right superior vena cava draining all the pulmonary venous blood

41. Infracardiac TAPVR

42. TAPVC - Infracardiac Lungs Descending vertical vein Liver heart

43. TAPVR - CXR Infracardiac = Obstructed = Surgical Emergency

46. Tricuspid atresia RA LA LV LA LV RV

49. Pulmonary atresia

52. Ebstein’s malformation of TV RA aRV RV aRV

55. Cyanosis and Hemoglobin