Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) associated with multiple large, coronary artery fistula represents a rare and complex congenital heart defect. The presence of Right Ventricular Dependent Coronary Circulation (RVDCC) critically influences management and prognosis. We report the case of a newborn with PA/IVS, extensive coronary fistulae involving both the right and left coronary arteries. A staged, stepwise approach was undertaken to exclude RVDCC, followed by transcatheter closure of the coronary fistulae and ballon perforation of the pulmonary valve. This sequential approach facilitated gradual right ventricular decompression and the establishment of antegrade pulmonary blood flow while minimizing the risk of coronary ischemia. Definitive biventricular circulation was subsequently achieved with placement of a right ventricle-to-pulmonary artery (RV-PA) conduit, resulting in right ventricular pressures decreasing to approximately onehalfsystemic levels.This case demonstrates the feasibility and effectiveness of a staged, multidisciplinary hybrid approach in carefully selected patients with PA/IVS and complex coronary artery fistulae. It underscores the importance of comprehensive coronary assessment and individualized management in optimizing outcomes in this challenging congenital cardiac anomaly.
Danner et al. (Thu,) studied this question.