A seventeen-year-old male was referred with fatigue on effort and cyanosis. On examination, he was cyanotic and had a systolic murmur at the left sternal border. Echocardiography showed normal situs, a large subaortic ventricular septal defect, tricuspid atresia, pulmonary artery stenosis, and a normal right ventricle. (Figs. 1 and 2). Both arteries arose exclusively from the left ventricle. The pulmonary artery was positioned posterior and to the left, and the aorta was positioned anterior and to the right. Cardiac catheterization revealed a pulmonary artery pressure of 41/18 mmHg (mean, 30) and arterial saturation of 91%. The patient was not believed to be suitable for single ventricle pallation.
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