Backer C L, Stout M J, Zales V R, Muster A J, Weigel T J, Idriss F S, Mavroudis C
Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Northwestern University Medical School, Chicago, IL 60614.
J Thorac Cardiovasc Surg. 1992 Jun;103(6):1049-57; discussion 1057-8.
Children with anomalous origin of the left coronary artery from the pulmonary artery are at risk for myocardial infarction and death. Surgical management of this condition in children has evolved significantly during the past 20 years. Between 1970 and 1990, a total of 20 of these patients underwent surgical intervention at two institutions. Age at operation ranged from 3 weeks to 11 years (mean, 26 months). Twelve patients had congestive heart failure, three were in cardiogenic shock, and two had cardiac murmurs. Operative techniques included ligation (n = 9), subclavian artery anastomosis (n = 5), aortic implantation (n = 3), internal mammary artery anastomosis (n = 1), intrapulmonary tunnel from aortopulmonary window to coronary artery (n = 1), and cardiac transplantation (n = 1). The three deaths in the series occurred at 3 weeks, at 2 months, and at 9 years after ligation. There have been no deaths after establishment of a two coronary artery system or after transplantation. Two of the five patients who had subclavian artery anastomosis to the anomalous coronary artery have severe anastomotic stenosis and collateralization. For patients with anomalous origin of the left coronary artery from the pulmonary artery, we recommend direct aortic implantation of the anomalous coronary artery at the time of diagnosis. Intrapulmonary tunnel from aortopulmonary window to coronary artery, or aorta-coronary bypass with internal mammary artery are recommended for children in whom aortic implantation is not anatomically feasible. Left coronary artery ligation is not indicated for these patients; those who have survived ligation should be considered for elective establishment of a two coronary artery system because of the risk of late death.
左冠状动脉起源于肺动脉的儿童有发生心肌梗死和死亡的风险。在过去20年中，儿童这种疾病的外科治疗有了显著进展。1970年至1990年期间，共有20例此类患者在两家机构接受了手术干预。手术年龄从3周龄至11岁（平均26个月）。12例患者有充血性心力衰竭，3例处于心源性休克，2例有心脏杂音。手术技术包括结扎（n = 9）、锁骨下动脉吻合术（n = 5）、主动脉植入术（n = 3）、胸廓内动脉吻合术（n = 1）、从主肺动脉窗到冠状动脉的肺内隧道术（n = 1）和心脏移植术（n = 1）。该系列中的3例死亡分别发生在结扎术后3周、2个月和9年。在建立双冠状动脉系统后或移植后均无死亡病例。5例接受锁骨下动脉与异常冠状动脉吻合术的患者中有2例有严重的吻合口狭窄和侧支循环形成。对于左冠状动脉起源于肺动脉的患者，我们建议在诊断时将异常冠状动脉直接植入主动脉。对于解剖上无法进行主动脉植入的儿童，建议采用从主肺动脉窗到冠状动脉的肺内隧道术或胸廓内动脉主动脉冠状动脉搭桥术。这些患者不适合进行左冠状动脉结扎术；由于存在晚期死亡风险，对于结扎术后存活的患者，应考虑择期建立双冠状动脉系统。
