Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA): Repair by Translocation
A three-week-old, 2.5 kg neonate who presented with heart failure symptoms and a systolic murmur was found to have anomalous origin of the left main coronary artery from the pulmonary artery. An echocardiogram showed severe left ventricular dysfunction with a dilated left ventricle and moderate to severe mitral valve regurgitation. The decision was made to proceed with urgent surgical repair.
Through a standard median sternotomy and aortic and single right atrial cannulation, cardiopulmonary bypass was initiated without difficulty. The ductus arteriosus was then ligated. The main pulmonary artery was separated from the aorta, and both branch pulmonary arteries were dissected and encircled with vessel loops. Both branch pulmonary arteries were temporary clipped and cardioplegia was administered simultaneously through the aortic and pulmonary root.
The main pulmonary artery was then transected above the sinotubular junction, and the ostium of the left coronary artery was visualized. The left main pulmonary artery was harvested with enough button and was mobilized off the epicardial surface of the heart. It was then translocated to the ascending aorta in a newly created ostium and was implanted using running 7-0 Prolene sutures. Cardioplegia administration through the aortic root confirmed the position and course of the translocated left main coronary artery to be satisfactory. The harvest site in the pulmonary root was then reconstructed with a decellularized pulmonary homograft patch. The heart was then deaired, and the aortic cross-clamp was removed. The patient regained his normal sinus rhythm, and the team completed the main pulmonary artery reconstruction on the beating heart while rewarming back to normothermia.
The vessel loops around the branch pulmonary arteries were then removed, and the patient was ventilated and subsequently weaned off cardiopulmonary bypass without difficulty. An epicardial echocardiogram showed good flow in the left main coronary artery. However, the left ventricle remained dilated with depressed function. All cannulae were then removed and heparin was reversed, followed by chest closure in the standard fashion.
The postoperative course was overall uneventful, and the patient was discharged eighteen days later. His left ventricular function continued to improve in the postoperative period and during follow-up with appropriate medical therapy. A six month follow-up echocardiogram showed trivial mitral valve regurgitation and normal left ventricular function and size.
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