We present a case of left isomerism with total anomalous systemic venous connection where the inferior vena cava was absent and all other systemic veins connected abnormally to the left atrium. The right atrium was hypoplastic with an intact atrial septum. Blood flow to the lungs was through a large ventricular septal defect. The diagnosis was made with echocardiography, angiography, and computed tomography. Complete repair was performed successfully, and the 7-year-old patient had an uneventful recovery.
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