[Double-outlet right ventricle with mitral atresia. Study of 9 cases]

Double-outlet right ventricle with mitral atresia is an uncommon anomaly with a few cases reported in the literature. We present 9 cases of this malformation that have been diagnosed by two-dimensional echocardiography (7 cases), cardiac catheterization (9 cases) and anatomical study (2 cases). We classify them into two groups according to whether or not they have associated pulmonary stenosis. The dominant symptoms were cyanosis and hypoxemia in the first group and cardiac insufficiency signs in the other. The left ventricle was hypoplastic in eight and normal in the one with tricuspid overriding. Six of the cases had ventricular septal defect. The great arteries were in normal relationship in 4 cases, with D-malposition in 3 cases and side-by-side in 2 cases. Rashkind atrial septotomy was performed in 5 patients, but was effective only in two. Palliative surgical treatment was performed on six of them. The actual survival rate is 44%.