Double outlet right ventricle with 1-malposition of the aorta

Four patients are described with a recently recognized variant of double outlet right ventricle. Clinical examination favoured tetralogy of Fallot, but the chest X-ray suggested corrected transposition. Catheterization and angiocardiography showed that the aorta was to the left of the main pulmonary artery, and both arose from a normally positioned morphological right ventricle. Egress of blood from the left ventricle was through a subaortic ventricular septal defect. In all patients severe pulmonary stenosis was present and the right coronary artery ran an anomalous course anterior to the pulmonary valve ring. Two children had successful total correction, and one a palliative Blalock-Taussing shunt. Necropsy material from the fourth patient allowed confirmation of the ventricular morphology and the conducting tissued was examined. In corrective surgery, blood from the left ventricle was rerouted into the aorta by an intraventricular baffle. Pulmonary stenosis was relived by infundibulectomy and outflow tract patch.