The innominate vein usually courses anterior to the aortic arch, where it joins the right brachiocephalic vein to form the superior caval vein. A retroaortic innominate vein is an uncommon finding in patients with congenital heart disease. We report a patient with a single ventricle, single atrium, pulmonary atresia, nonconfluent pulmonary artery, persistent left superior caval vein, absent inferior caval vein (azygos connection), right aortic arch, and retroaortic innominate vein. His innominate vein took an anomalous course. The right brachiocephalic vein crossed from right to left underneath the aortic arch and formed a left superior caval vein with the left brachiocephalic vein and the azygos vein. We reconstructed the nonconfluent pulmonary artery using the retroaortic innominate vein and then performed a total cavopulmonary shunt when he was 32 months of age. Thereafter, a total cavopulmonary connection was carried out at age 42 months.

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