Double-outlet right ventricle without interventricular communication: an unusual and challenging problem.

A ventriculoseptal defect (VSD) usually is present in patients with a double-outlet right ventricle. This report describes a case of double-outlet right ventricle with a completely shrouded inlet VSD and no interventricular shunting. This was associated with a severely regurgitant dysplastic mitral valve. The anatomy and clinical management of the patient is discussed together with an overview of the existing literature.