Congenital Heart Disease Cheston M. Berlin, Jr., M.D. Department of Pediatrics

Stephen Cyran, M,D

Congenital Heart Disease Ask two questions: –Is the patient cyanotic (blue)? –Is a shunt (blood flow) present? Occurs in 1% of newborns Most can be fixed Patients need to be followed into adult life

Congenital Heart Disease SHUNT CYANOSIS

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Pulmonary artery Aorta NORMAL HEART

Atrial Septal Defect - ASD O.5 per thousand births Patent foramen ovale - has flap, only right to left shunt. Must exist for life to continue in some defects. For both Ostium Secundum and Ostium Primum - increased L R shunt Surgical closure effective

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Pulmonary artery Aorta Atrial Septal Defect

Ventricular Septal Defect - VSD 2.2 per 1,000 births Small lesions very common - 3-5% - spontaneous closure Increased pulmonary blood flow - may lead to pulmonary hypertension Surgical closure effective If high in ventricular wall, will involve the atria - endocardial cushion defect

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Pulmonary artery Aorta Ventricular Septal Defect

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Pulmonary artery Aorta AV Canal (endocardial cushion defect)

Coarctation of the Aorta 0.3 per 1,000 births May be silent - hypertension in the upper extremities is the clue Surgery effective

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Pulmonary artery Aorta Coarctation of the aorta

Stephen Cyran, M,D.

a o r t a Coarctation (post ductal) Left ventricle

Patent Ductus Arteriosus 30-40% in premature infants 0.4 per 1,000 in term infants May cause heart failure in the newborn period Presence may be life saving other cardiac defect prevents L to R shunt Surgery routine - ligation

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Pulmonary artery Aorta Patent ductus arteriosus

Conotruncus Most distal portion of the primitive heart tube Determines normal separation of the aorta and pulmonary outflow vessels Determines their normal alignment with the ventricles

Conotruncus, Cont’d Proliferation of cells within the conotruncal cushion Migration of cells into the conotruncus from the cardiac neural crest Resorption of the subaortic conus Leftward movement of the conotruncus

Truncus Arteriosus Common outlet vessel VSD always present permitting mixing of pulmonary with systemic blood.

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Truncus Truncus arteriosus

Transposition of the Great Vessels Complex group of lesions The pulmonary artery arises from the left ventricle, and the aorta from the right ventricle Incompatible with life unless shunting occurs to permit oxygenated blood to reach the systemic circulation Surgery difficult, but effective

svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Pulmonary artery Aorta Transposition of the great vessels

Tetralogy of Fallot 0.5 per 1,000 live births Overriding aorta Ventricular Septal Defect Pulmonary stenosis Hypertropied wall of right ventricle May need temporary shunt before total surgical correction

Pulmonary artery svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 right 2 left Aorta Tetralogy of Fallot

Total Anomalous Pulmonary Venous Return O.1 per 1,000 births All pulmonary venous blood returns to the right side of the heart, usually the right atrium Must have shunt for survival 30% of patients have other lesions Surgery may be complex: depends on anatomy

Pulmonary artery svc ivc Pulmonary veins Right atrium Left atrium Right ventricle Left ventricle 2 left Aorta Total anomalous pulmonary venous return 2 right Normal site

TGV Pulmonary atresia