先天性冠状动脉起源异常病理解剖及胚胎学与外科治疗

Pathological anatomy,Embryology and surgical treatment of Congenital Anomalous Origin of Coronary Artery

目的总结先天性冠状动脉起源异常病理解剖和胚胎学与外科治疗。方法回顾分析阜外医院1998年2月到2007年8月先天性冠状动脉起源异常并进行外科手术病例。其中24例患者冠状动脉起源于肺动脉。男13例。女11例；年龄3月到41岁。左冠状动脉起自肺动脉14例；右冠状动脉起自肺动脉10例。按Leiden冠状动脉分型方法进行冠状动脉分型。左冠状动脉起自肺动脉14例均在体外循环下进行手术，直接冠状动脉移植12例，肺动脉内隧道2例。右冠状动脉起自肺动脉10例中2例在非体外下进行冠状动脉移植，1例合并房间隔缺损进行房间隔封堵术，2例进行肺动脉内隧道，6例进行了直接移植。1997年10月--2007年8月共完成动脉掉转手术170例。采用Leiden冠状动脉分型方法，1LCx2R为冠状动脉正常分布，其余分类形式为冠状动脉异常分布。冠状动脉异常起源48例，有3例冠状动脉壁内走行；男28例，女20例，年龄4天～6岁；简单型大动脉转位中18倒冠状动脉异常起源，复杂型大动脉转位中15例冠状动脉异常起源，T-B中15例冠状动脉异常起源。冠状动脉吻合主要采用带片开门技术或吊窗技术。结果冠状动脉起源乎肺动脉病例中，左冠状动脉均起自肺动脉窦1，右冠状动脉均起自肺动脉窦2；异常肺动脉起源的冠状动脉冠状动脉再移植无手术死亡。动脉掉转手术冠状动脉异常起源48例中，其中1L2RCx占18例、1R2LCX7例、1LR2Cx6例、2LCx2R6例、2LCxR5例。异常主动脉起源的冠状动脉冠状动脉再移植死亡9例，死亡率为18．7％。其中2004年前死亡7例，死亡率29．2％（7／24），2004年后死亡2例，死亡率8．3％（2／24）。结论提高对先天性冠状动脉起源异常的病理解剖，胚胎发生学的认识有助于提高手术疗效。

Objective To summarize pathological anatomy, embryology and surgical results of Congenital Anomalous Origin of the Coronary Artery. Methods From February, 1998 to August, 2007, 24 patients, 13 males and 11 femals with ages ranging from 3 month old to 41 years old with anomalous origin of the coronary artery from the pulmonary artery underwent surgical correction. There were 14 patients with anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA）, and 10 patients with anomalous origin of the right coronary artery from the pulmonary artery（ARCAPA）. Eight patients underwent direct aortic reimplantation, and 4 patients underwent a Takeuchi procedure （intrapulmonary artery baffle with an autologous pericardial patch）. One patient of ARCAPA with atrial septal defect（ASD） was closed with an amplatzer septal occluder. From October, 1997 to December, 2006, 170 patients underwent arterial switch operations. The mean age ranged from 4 days to 6 years. We used Leiden classification for coronary artery pattern by operative descriptions. The most prevalent coronary pattern was [ 1LCx2R], the others were abnormal coronary artery pattern of 48 cases（48/170, 28.2% ）, and intramural course of coronary artery was found in three patients . In most patients , we used the ＂ open trap door ＂ or ＂bay window＂ technique for coronary ostia transfer. Results In ALCAPA, The anomalous origin of the left coronary artery all came from sinus I of pulmonary artery. In ARCAPA, the anomalous origin of the right coronary artery all came from sinus 2 of pulmonary artery. There were no deaths with anomalous origin of the coronary artery from the pulmonary artery. In ASO cases, the abnormal coronary artery pattern included 1L2RCx 18 cases, 1R2LCx 7 cases, 1LR2Cx 6 cases, 2LCx2R 6 cases and 2LCxR 5 cases. There were 9（18.7% ） hospital deaths of ASO with abnormal coronary artery pattern, 28 % hospital deaths before 2004 and 8.3 % hospital deaths after 2004. Conclusions To enhance understanding of the pathological anatomy, embry- ology of congenital anomalous origin of the coronary artery may improve the results of surgery.