Pulmonary Atresia With Intact Ventricular Septum: Expert Review of the Surgical Management

Abstract

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare but highly heterogeneous congenital heart lesion defined by absence of continuity between the right ventricle (RV) and pulmonary arteries, a morphologically variable tricuspid valve (TV), RV hypoplasia and frequent right ventricle to coronary connections. Management has evolved from uniform surgical palliation toward nuanced, morphology-driven individualized strategies. Current approaches include transcatheter perforation, surgical valvotomy, staged ventricular recruitment, and single-ventricle or 1.5-ventricle palliation, with transplantation reserved for select patients. Recent publication reported a successful procedure to palliate coronary RV dependency with an aortic to TV conduit. Long-term outcomes hinge on RV growth potential, coronary anatomy, and institutional expertise.