Double Outlet Right Ventricle with Pulmonary Atresia and Antero-Posterior Malposition of Great Arteries: Transthoracic Echocardiographic Evaluation of a rare cyanotic congenital heart disorder

Abstract

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle, This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We are reporting a case of seven year old deeply cyanotic child with DORV, subaortic VSD with rare anatomic features of antero-posterior malposition of great arteries (GA) and pulmonary vavular atresia, with conspicuous absence of patent ductusarteriosus (PDA) or major aorto-pulmonary collaterals (MAPCAs), despite the presence of atretic pulmonary valve. Keywords: DORV, Pulmonary atresia, TGA, VSD, subaortic VSD, overriding of Aorta, cyanotic congenital heart defects.