Isolated right ventricular hypoplasia with atrial septal defect

Abstract

Isolated Hypoplasia of the right ventricle is a rare congenital cardiac anomaly which has been infrequently reported in the literature. Less than fifteen cases have so far been recorded. A description of the clinical, hemodynamic and angiographic findings in two cases is presented. Both the cases reported here presented with a clinical picture of tricuspid atresia. In both cases the correct diagnosis was suspected during cardiac catheterization from the characteristics of the pressure tracings. The angiocardiographical appearances in both cases excluded malposition of the tricuspid valve; the size of the cavity of the right ventricle was compatible with hypoplasia of the muscle wall rather than the total aplasia first described by UhI.