Extremely late presentation of children with transposition the great arteries.

We present three cases of primary arterial switch operation for extremely late presenting transposition the great arteries with intact ventricular septum: a 7-year-old female, 3-year-old male, and 6-year-old female. Two patients were discharged on postoperative day 9 and 11, the other developed hemodynamic instability 12 h after surgery and died due to left ventricular failure and pulmonary hypertension. Left ventricular mass index >35 g·m-2 and left ventricular posterior wall >4 mm are criteria for a primary arterial switch operation. Circulatory support post- or intraoperatively might provide better results. Two patients survived without extracorporeal membrane oxygenation or nitric oxide.