Tavakoli Reza, Jamshidi Peiman, Yamani Nassrin, Gassmann Max
Institute of Veterinary Physiology and Zurich Center of Integrative Human Physiology (ZIHP), University of Zurich;
Department of Cardiology, Canton Hospital Lucerne.
J Vis Exp. 2017 Apr 24(122):55590. doi: 10.3791/55590.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly which is one of leading causes of myocardial ischemia and infarction in children. If left untreated, it results in a 90% mortality rate in the first year of life. In patients who survive to the adulthood, the coronary steal phenomenon and retrograde left-sided coronary flow provide a substrate for chronic subendocardial ischemia, which may lead to left ventricular dysfunction, ischemic mitral regurgitation, malignant ventricular arrhythmias, and sudden cardiac death. The average age of life-threatening presentation is 33 years and of sudden cardiac death 31 years. Therefore, surgical correction is highly recommended as soon as the diagnosis is made, regardless of age. In adult-type ALCAPA originating from the right-facing sinus of the pulmonary artery, direct re-implantation of the ALCAPA into the aorta is the more physiologically sound repair technique to re-establish the dual-coronary perfusion system and is recommended. This protocol describes the technique of direct re-implantation of adult-type ALCAPA into the aorta.
左冠状动脉起源于肺动脉（ALCAPA）是一种罕见的先天性异常，是儿童心肌缺血和梗死的主要原因之一。若不治疗，在生命的第一年死亡率达90%。存活至成年的患者，冠状动脉窃血现象和左侧冠状动脉逆行血流为慢性心内膜下缺血提供了基础，这可能导致左心室功能障碍、缺血性二尖瓣反流、恶性室性心律失常和心源性猝死。危及生命表现的平均年龄为33岁，心源性猝死的平均年龄为31岁。因此，一旦确诊，无论年龄大小，强烈建议进行手术矫正。对于起源于肺动脉右窦的成人型ALCAPA，将ALCAPA直接重新植入主动脉是重建双冠状动脉灌注系统更符合生理的修复技术，值得推荐。本方案描述了将成人型ALCAPA直接重新植入主动脉的技术。
