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Ann Thorac Surg 2001;71:1989
© 2001 The Society of Thoracic Surgeons
a Division of Cardiothoracic Surgery, University of Iowa Hospitals and Clinics, John Colloton Pavilion, Room 1602-A, 200 Hawkins Dr, Iowa City, IA 52242, USA
e-mail: douglas-behrendt{at}uiowa.edu
This series demonstrates the remarkable progress made in changing the outlook of an anomaly which was 95% fatal thirty years ago to one in which 16 of 17 patients survived. In the 1960s it was recognized that simple ligation of the left main coronary artery would eliminate "coronary steal" and transiently benefit those patients with sufficient collaterals from the right coronary to provide left ventricular perfusion. However, this did not help the majority of infants who had insufficient collaterals and who developed myocardial ischemia and mitral valve regurgitation from papillary muscle dysfunction and ventricular dilatation. Attempts were made at creating a two coronary circulation with interposed saphenous veins, prostheses, and subclavian arteries or by creating intrapulmonary tunnels. Mortality rates exceeded 75% and long-term patency was poor. In 1974 Netches and colleagues [1] described attaching the left coronary artery with a surrounding button of pulmonary artery directly to the aorta. This became the operation of choice and the prognosis for these patients has steadily improved. This transformation was facilitated by improvements in infant perfusion, experience with transferring coronary arteries during the arterial switch procedure, development of extracorporeal membrane oxygenation for postoperative left ventricular support, and advances in perioperative management.
Once the technique for correction became standardized, several questions remained:
Levitsky and colleagues [2] definitively demonstrated in 1980 that ventricular function could improve to normal, an observation confirmed by others including the present authors. It is remarkable how quickly this recovery occurs, within six months in the present report. Even in those patients who require extracorporeal membrane oxygenation only a few days of support are required. Important mitral regurgitation has been shown to regress without valvuloplasty in about 80% of cases. An area for future study is to determine criteria by which the remaining 20% can be identified and to determine if primary valvuloplasty or valve replacement in this subset will improve long-term results. A major new contribution of the present report is to point out that, when mitral regurgitation returns after initially improving, coronary artery obstruction should be suspected. While the patency rate of the transferred coronary arteries has been 100% in most series, there are occasional failures. Experience has shown that the anomalous coronary usually arises from the right-facing pulmonary sinus, making transfer possible without tension. In those few instances in which the origin is remote an alternative technique might preferably be chosen such as the creation of a tube from pulmonary artery flaps. The authors believe that the reported surgical failures represent technical imperfections. However, it must be remembered that congenital coronary stenoses do occur in anomalous left coronary arising from the left pulmonary artery (ALCAPA) and that these may only become apparent postoperatively.
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