Single ventricle defects, including inflow emptying into 1 ventricle, occur at an incidence of 0.05-0.1 per 10,000 live births. The association of total anomalous pulmonary venous drainage (TAPVD) with single ventricle defects is a risk factor for poor outcome. Conversely, common atrium, a rare congenital anomaly, is an endocardial cushion defect. We present a rare case of an unoperated patient with coincidence of a single ventricle defect, TAPVD with direct drainage from the pulmonary veins into the superior vena cava, and common atrium. To the best of our knowledge, such a case has not been previously reported in the literature.
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