Invited Commentary: The Fate of MAPCAs.

Abstract

Pulmonary valve atresia with ventricular septal defect (PA/VSD) rep-resents the most extreme form of tetralogy of Fallot. In contradistinc-tion to patients with tetralogy of Fallot, there is no continuity between the right ventricle and the pulmonary artery tree. Therefore, pulmonary blood supply in these patients is dependent on extracardiac sources. These sources may be the ductus arteriosus, collateral arteries from the thoracic aorta