Pulmonary Vascular Sequelae of Palliated Single Ventricle Circulation: Arteriovenous Malformations and Aortopulmonary Collaterals

Children and adults with single ventricle congenital heart disease (CHD) develop many sequelae during staged surgical palliation. Universal pulmonary vascular sequelae in this patient population include two inter-related but distinct complications: pulmonary arteriovenous malformations (PAVMs) and aortopulmonary collaterals (APCs). This review highlights what is known and unknown about these vascular sequelae focusing on diagnostic testing, pathophysiology, and areas in need of further research.