DefinePK

Abstract

ABSTRACTPulmonary atresia with intact interventricular septum(PA-IVS) is a rare congenitallesion. PA-IVS involves complete blockage of the pulmonary valve located on theright side of the heart. This blockage thus prevents the flow of blood to the lungs.Because of this lack of blood flowing through the right side of the heart, thestructures on that side, such as the pulmonary valve and the tricuspid valve, maybe abnormally small. The genetic cause of PA-IVS is unknown. We report a 6-month-old infant with congenital heart disease including; pulmonary atresia,intact ventricular septum, right ventricular hypoplasia, and vertical persistantductus arteriosus (PDA) arising from left subclavian artery with stenosis atpulmonary end. PDA stenting was performed with successful hemodynamicafter procedure.