CONGENITAL HEART DISEASE IN THE ADULT Classification: 1.

Simple Adult Congenital Heart Disease single lesions with a shunt or a valvular malformation a. Native disease i. Uncomplicated congenital aortic valve disease ii. Mild congenital mitral valve disease (e.g. except parachute valve, cleft leaflet) iii. Uncomplicated small atrial septal defect iv. Uncomplicated small ventricular septal defect v. Mild pulmonic stenosis b. Repair conditions i. Previously ligated or occluded ductus arteriosus ii. Repaired secundum or sinus venosus atrial septal defect without residua iii. Repaired ventricular septal defect with residua 2. Intermediate complexity CHD more than 2 or more simple defects a. Ostium primum or sinus venosus atrial septal defect b. Anomalous pulmonary venous drainage, partial or total c. Atrioventricular canal defects (partial or complete) d. Ventricular septal defect, complicated (e.g. absent or abnormal valves or with associated obstructive lesions, aortic regurgitation) e. Coarctation of the aorta f. Pulmonic valve stenosis (moderate to severe) g. Infundibular right ventricular outflow obstruction of significance h. Pulmonary valve regurgitation (moderate to severe) i. Patent ductus arteriosus (non-closed) moderate to large j. Sinus of Valsalva fistula aneurysm k. Subvalvular or supravalvular aortic stenosis 3. Complex CHD have components of an intermediate defect + more complex cardiac and vascular anatomy, often with cyanosis, and frequently with transposition complex a. Cyanotic congenital heart diseases (all forms) b. Eisenmengers syndrome c. Ebsteins anomaly d. Tetraology or Fallor or pulmonary atresia (all forms) e. Transposition of the great arteries f. Single ventricle; tricuspid or mitral atresia g. Double-outlet ventricle h. Truncus arteriosus i. Fontan or Rastelli procedures Diseases: 1. Atrial Septal Defect (ASD) common in female adults; true deficiency of the atrial septum and implies functional and anatomy patency; asymptomatic in early life a. Sinus venosus ASD occurs high in the atrial septum near the entry of the superior vena cava into the (R) atrium and is associated frequently with pulmonary venous connection from the (R) lung to the SVC or (R) atrium b. Ostium primum ASD lie adjacent to the AV valves (may be deformed and regurgitant); common in Down syndrome (more complex AV septal defects with a common AV valves and a posterior defect of the basal portion of the intraventricular septum are more typical in Downs) c. Ostium secundum ASD most common: fossa ovalis; midseptal in location; should not be confused with a patent foramen ovale i. Anatomic obliteration of the foramen ovale normally follows its functional closure soon after birth, but residual probe patency is normal d. L-to-R shunt: i. Magnitude depends on: 1. ASD size 2. Ventricular diastolic properties 3. Relative impedance in the pulmonary and systemic circulations ii. Causes: 1. Diastolic overloading of R ventricle 2. Increased pulmonary blood flow e. Physical examination: i. Prominent RV impulse ii. Palpable pulmonary artery pulsation iii. First heart sound: Normal or split iv. Second heart sound: widely split and relatively fixed in relation to respiration v. Mid-diastolic rumbling murmur, loudest at 4th intercostal space and along L sternal

vi.

vii. f. ECG: i.

border, reflects increased flow across tricuspid valve Ostium primum ASD apical holosystolic murmur indicates associated mitral or tricuspid regurgitation or a ventricular septal defect Cyanosis and clubbing accompany the development of a R-to-L shunt (VSD)

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9. 10. 11. 12. 13. 14. 15.

Ostium secundum ASD R-axis deviation and rSr pattern in R precordial leads g. Chest X-Ray: i. Enlarged R atrium and R ventricle, and pulmonary artery and its branches ii. Increased pulmonary vascular markings of L-to-R shunt vascularity will diminish if pulmonary vascular disease develops h. Treatment: i. Operative repair patch of pericardium/prosthetic material or percutaneous transcatheter device closure for uncomplicated secundum ASD with significant L-to-R shunting (pulmonaryto-systemic flow ration >=2:1) ii. Ostium primum ASD cleft mitral valves may require repair in addition to patch closure of the ASD Ventricular Septal Defect Patent Ductus Arteriosus Aortic Root-to-Right Heart Shunts Congenital Aortic Stenosis a. Subaortic Stenosis b. Supravalvular Aortic Stenosis Coarctation of the Aorta Pulmonary Stenosis with Intact Ventricular Septum Tetralogy of Fallot a. Four components: i. Malaligned VSD ii. Obstruction to RV outflow iii. Aortic override of the VSD iv. RV hypertrophy d/t RVs response to aortic pressure via the large VSD Complete Transposition of the Great Arteries Single Ventricle Tricuspid Atresia Ebsteins Anomaly Congenitally corrected transposition Malpositions of the heart (dextro, meso, levocardia) Endocarditis prophylaxis