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ALCAPA with the ectopic orifice at the non-facing sinus: successful anatomic repair by creation of an autologous extrapulmonary tunnel.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect. This anomaly leads to a coronary hypoperfusion phenomenon and to substantial left ventricular dysfunction caused by abnormal perfusion of the left ventricle. The optimal surgical management of such cases is not clearly established. Here, we report the successful anatomic repair of ALCAPA arising from the non-facing sinus of Valsalva of the pulmonary artery in a 5-kg patient. In order to perform the repair, we created an autologous extrapulmonary tunnel (from a pulmonary artery flap and autologous pericardium), which we implanted into the ascending aorta. Because of post-cardiotomy heart failure, we implanted an extracorporeal membrane oxygenation device during the same procedure. After recovery of the failing heart, the device was easily ex-planted, and the patient was discharged from the hospital on postoperative day 30.