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Double outlet right ventricle with intact ventricular septum

We report a rare case of DORV with an intact ventricular septum (intact VS). The patient was a 2-day-old boy, born at 39 weeks and weighing 3090 g, who was referred to the Department of Pediatrics, Sapporo Medical University Hospital, Sapporo, Japan, because of a heart murmur. He had cyanosis and a systolic murmur at the lower intercostal spaces. Chest X-ray showed cardiomegaly (cardiothoracic ratio, 67%) and the electrocardiogram revealed normal sinus rhythm, right axis deviation and right ventricular hyper trophy. Echo cardiography demonstrated situs solitus, normal relationship of great arteries, side by side, bilateral conus, intact VS, persistent foramen ovale, pulmonary stenosis and severe mitral valve insuffi ciency ( Fig. 1a,b). We performed balloon atrial septostomy when he was 1 month old, and he underwent a left modifi ed Blalock – Taussig shunt at 5 months. Cardiac catheterization was performed when he was 18 months old. His left ventricular pressure was extremely low, 10/4 mmHg, the diastolic volume was 11 mL, 48% of normal, and the ejection fraction was 76%. Left ventricular angiography showed a smooth and thin wall of the left ventricle, resembling Uhl’s disease. Both right and left coronary artery were normally demonstrated ( Fig. 2). Right ventricular pressure was 92/11 mmHg with a pulmonary artery Patient Report