Patients with right atrial isomerism and total anomalous pulmonary venous connection (TAPVC) in a functionally univentricular heart are a challenging subset with a high mortality rate. We present the case of a patient with univentricular heart who had right atrial isomerism and associated mixed TAPVC (supracardiac, cardiac, and infracardiac). The anatomy was delineated precisely, and the patient underwent first-stage univentricular palliation consisting of TAPVC repair and bilateral bidirectional superior cavopulmonary anastomosis.
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