儿童冠状动脉起源于肺动脉16例手术治疗分析

Anomalous origin of the coronary artery from the pulmonary artery in 16 children

目的探讨儿童冠状动脉起源于肺动脉的外科治疗方法及疗效。方法冠状动脉起源于肺动脉患儿16例,其中左冠状动脉起自肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)14例,右冠状动脉起自肺动脉(anomalous origin of the right coronary artery from the pulmonary artery,ARCAPA)2例,均行冠状动脉起源异常矫治手术,13例异常起源冠状动脉与升主动脉直接移植吻合,3例行Takeuchi术;对4例合并二尖瓣重度反流者行二尖瓣成形术,1例左心室室壁瘤行室壁瘤切除。术后随访6个月观察治疗效果。结果本组无围手术期死亡病例,住院期间无并发症发生;术后6个月时,患儿心胸比(0.50±0.01)、左心室舒张末期内径[(33.4±4.3)mm]较术前[0.57±0.08、(39.6±8.1)mm]明显减小(P<0.05),左室射血分数[(0.66±0.07)%]、短轴缩短率(0.37±0.01)较术前[(0.57±0.15)%、(0.31±0.09)]明显增加(P<0.05),二尖瓣轻度反流5例。结论双冠状动脉解剖矫治是治疗冠状动脉起源于肺动脉的常用方法,近期疗效良好。

Objective To summarize the surgical treatment and outcome of anomalous origin of the coronary artery from the pulmonary artery in children. Methods In 16 cases of anomalous origin of the coronary artery from the pulmonary artery, there were 14 cases of anomalous origin of the left coronary artery from the pulmonary artery and 2 cases of anomalous origin of the right coronary artery from the pulmonary artery. All received surgical correction operation, including direct transplantation for anomalous origin of coronary artery and ascending aorta in 13 cases and Takeuchi operation in 3 cases. Four cases complicated with severe mitral regurgitation accepted mitral valvuioplasty and one case of ventricular aneurysm received resection. All patients were followed up for 6 months. Results No perioperative death and no complications during hospitalization occurred. Six-month follow-up result showed cardiothoracic ratio （0.50± 0.01） was smaller, left ventricular end-diastolic dimension （（33.4 ± 4.3） mm） was shorter, left ventricular ejection fraction （（0.66±0.07）%） was larger, and short axis shortening fraction （0. 37±0.01） was larger than those before operation （（0. 57±0.08） %, （39.6±8.1） mm, 0. 57±0.15, 0. 31±0.09）） （P〈0.05）. Slight mitral valve regurgitation occurred in 5 cases. Conclusion Double coronary artery anatomical correction is a common method for the treatment of anomalous origin of the coronary artery from the pulmonary artery, with a good short-term effect.