Coexistence of Single Ventricle with Atresia of One Atrioventricular Orifice

A new case of an unusual association of mitral atresia with single left ventricle is reported. The salient anatomic features include: (1) the right atrioventricular valve is atretic; (2) the left atrioventricular valve enters the large ventricle with morphologic characteristics of the left ventricle; and (3) this large ventricle communicates by way of an interventricular communication with a smaller left-sided ventricle, but with the morphologic characteristics of the right ventricle. Guidelines for the recognition of these uncommon malformations are given.