Stenosing Supravalvular Ring of the Left Atrium Associated With Atrioventricular Discordance and Double Outlet Right Ventricle (SLD)

Abstract

We experienced a rare cardiac anomaly associated with atrioventricular discordance, double outlet right ventricle (SLD), ventricular septal defect, stenosing supravalvular ring of the left atrium and hypoplasia of the tricuspid valve and right ventricle. The patient did not survive the operation which consisted of resection of the supravalvular ring, Senning procedure and intraventricular rerouting with a conduit. Autopsy findings suggested that hypoplasia of the tricuspid valve and the right ventricle would be related to the patient's death. Recognition of the presence of hypoplasia of the tricuspid valve and the right ventricle is much important for surgical treatment.