Echocardiographic features of double-outlet right atrium and straddling tricuspid valve with intact ventricular septum: a rare cardiac anomaly associated with pulmonary atresia and single coronary artery ostium.

Double-outlet right atrium is a rare congenital cardiac anomaly previously described in association with atrioventricular canal defect. Straddling tricuspid valve is another uncommon anomaly that is almost always associated with a ventricular septal defect. We report the echocardiographic features of a newborn with double-outlet right atrium associated with pulmonary atresia, intact ventricular septum, and right coronary artery ostial atresia. Alternatively, the anatomy can be interpreted as straddling tricuspid valve with intact ventricular septum. The echocardiographic findings were confirmed by cardiac catheterization. 2010 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.