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At the Mayo Clinic, four patients were seen in whom a ventricular septal defect (VSD) of the atrioventricular (A-V) canal type was associated with an angle of nearly 90° between the intact atrial septum and the ventricular septum. The right atrium overrode the ventricular septum and communicated with both ventricles. A complex, straddling right A-V valve had some attachments in the left ventricle and resembled the common A-V valve of persistent common A-V canal. In three of the patients, a congenital mitral valve deformity also existed. Mild cyanosis probably is not a contraindication to surgical repair in such patients because right atrial blood may enter the left ventricle directly.

Ventricular Septal Defect of Atrioventricular Canal Type with Straddling Right Atrioventricular Valve and Mitral Valve Deformity