Univentricular heart

Abstract

A univentricular heart, or univentricular atrioventricular (AV) connection (UAVC), is not a single lesion but a broad term encompassing congenital malformations leading to a single ventricular physiology, a very rare condition where one dominant ventricle supports both circulations. Tricuspid atresia is the classic example and the most common variety of UAVC. Other varieties include double-inlet right or left ventricle, mitral atresia, hypoplastic left heart syndrome, unbalanced common AV canal, pulmonary atresia, and heterotaxy syndromes. Heterotaxy syndromes refer to disorders of lateralization whereby the arrangement of abdominal and thoracic viscera differ from normal and mirror image of normal (Khairy et al., Circulation 115:800-812, 2007).