We report an adult case of right aortic arch coarctation with a left descending aorta. Associated anomalies included dextrocardia, situs inversus, polysplenia, bilateral superior vena cava, and absent inferior vena cava with azygos connection. Extensive mobilization of the azygos vein was needed to obtain a good surgical field, including the left descending aorta via a right thoracotomy, and the lesion was anatomically repaired by resection and end-to-end anastomosis.
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