Structural congenital heart defect Definition Left coronary Anomalous Left main Coronary Artery arises from the artery anomalies Pulmonary Artery rather than the aortic root (ALCAPA) Desaturated blood with low coronary perfusion pressure compromises blood flow as PVR drops in the newborn This and other variations of the coronaries may present as sudden death or CHF Aortic regurgitation Diastolic regurgitation of blood from the aorta back into (AR) the left ventricle Aortic stenosis Three types: subvalvar, valvar, and supravalvar (AS) Subtotal narrowing/obstruction of the left ventricular outflow at or near the aortic valve Aortopulmonary Defect between the aorta and pulmonary artery window Pulmonary overcirculation and left-sided volume overload Atrial septal defect A communication between the right and left atria (ASD) Usually asymptomatic Causes right-sided volume overload Atrioventricular Incomplete fusion of the embryonic endocardial cushions septal defect resulting in defects of the lower atrial septum (primum (AVSD) ASD), inlet ventricular septum, and atrioventricular valves, in isolation or combination Bicuspid aortic Aortic valve composed of two cusps, or three cusps with valve (BAV) two commissures yielding two effective cusps Congenital mitral Mitral valve leaflets are thickened and/or fused, producing stenosis a narrowed opening from the left atrium to the left ventricle, may be associated with an abnormal papillary muscle (parachute MV) Coarctation of the A discrete narrowing of the distal segment of the aortic Aorta (CoA) arch, typically just distal to the left subclavian artery at the point of ductal insertion Cor triatriatum The pulmonary veins join in a confluence that is not completely incorporated into the left atrium Double-outlet right The majority of the semilunar valve orifices of both great