Managing a rare Major Aortopulmonary Collaterals with Single Ventricle Physiology through Coiling and Cavopulmonary shunt in a single hybrid setting

Abstract

Double Outlet Right Ventricle (DORV) occurs in 3–9 per 100,000 live births.¹ Major Aortopulmonary Collateral Arteries (MAPCAs) are a rare component of congenital heart defects (CHDs), often complicating conditions like pulmonary atresia with ventricular septal defect (PA/VSD).² The Bidirectional Glenn (BDG) procedure establishes a superior Cavo pulmonary anastomosis in patients with single ventricle physiology (SVP).³ We report a case of a 15-year-old female with SVP, DORV, situs inversus, and MAPCAs, treated in a single hybrid setting with endovascular coiling of nonessential MAPCAs and BDG procedure. Current guidelines recommend optimizing the pulmonary vascular bed through unifocalization of essential MAPCAs and embolization of nonessential MAPCAs to achieve Cavo pulmonary anastomosis, often via single- or multi-staged approaches.^4,5 While MAPCA management in PA/VSD is well-documented, its management in SVP remains underexplored.⁵ This case demonstrates the feasibility of a hybrid approach for complex CHDs.