Unilateral Pulmonary Artery Agenesis with Right Sided Aortic Arch

Abstract

Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly due to a malformation of the sixth aortic arch during embryogenesis. It can occur as isolated or may be associated with other congenital cardiovascular malformations. Right-sided aortic arch (RAA) is infrequently seen entity that can be related to various forms of congenital heart disease. Among these, with mirror-image branching of the major arteries is the most encountered subtype. We have presented here a rare case of UPAA with RAA and Kommerell's diverticulum.