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Pulmonary Atresia With Intact Ventricular Septum: Expert Review of the Surgical Management
Charles D Fraser1, Davi F Tenorio2, Lee M Fuentes3
1Cardiovascular and Thoracic Surgery, Dell Children's Medical Center, Texas Center for Pediatric and Congenital Heart Disease, University of Texas at Austin Dell Medical School, Austin, Texas.
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.
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