Doublet outlet right ventricle associated with persistent common atrioventricular canal

Abstract

A variant of double outlet right ventricle (DORV) associated with common atrioventricular (A-V) canal was identified. As yet, this syndrome has not been successfully repaired. Of 16 patients with this diagnosis, 14 had the diagnosis confirmed at postmortem examination and 2 had the diagnosis confirmed by exploratory cardiotomy. The 16 patients were divided into 2 groups according to the position of the cardiac apex: group 1, 10 patients with levocardia, and group 2, 6 patients with dextrocardia and ventricular inversion. When pulmonary stenosis was present, the clinical presentation was similar to that in tetralogy of Fallot, and when such stenosis was not, the presentation was similar to that in ventricular septal defect with bidirectional shunting. The electrocardiogram had the typical feature of common A-V canal. The right ventricular angiocardiogram had features typical of DORV. The left ventricular angiocardiogram revealed left ventricular outflow deformity typical of A-V canal in patients with levocardia but not in those with dextrocardia. Splenic anomalies were commonly seen (9 out of the 16 patients). Asplenia was present in 7, and polysplenia in 10. 10 Patients had persistent left superior vena cava, 7 had common atrium, 6 had anomalous pulmonary venous connection, and 3 had subaortic stenosis. 12 of the 16 patients had pulmonary stenosis. Of the 16, 14 had common A-V canal (Rastelli type C) and 2 had a ventricular septal defect of the persistent common A-V type.