An Unusual Presentation of Double-Outlet Right Atrium.

We describe an unusual example of double-outlet right atrium with separate atrioventricular junctions. The straddling and overriding tricuspid valve had two orifices, and the mitral valve was morphologically normal. An appropriate understanding of the morphology of the atrioventricular junctions, the valves, and the subvalvar apparatus, along with the location of the atrioventricular conduction axis, allowed for successful biventricular repair.

Anomalous systemic venous connection to left atrium in a heart with usual atrial arrangement.

Anomalous systemic venous connection to left atrium is rare anomaly. Previously published cases described this anatomy in patients with left isomerism. Depending on the size of the atrial septal defect, patients usually present with varying degrees of cyanosis and right heart hypoplasia.