左冠状动脉异常起源于肺动脉合并左冠状动脉壁内走行的外科治疗  

Surgical treatment of patients with anomalous left coronary artery from the pulmonary artery and intramural left coronary artery

作　　者：顿耀军 张昌伟 刘锐 马凯 芮璐 张本青 林野 李守军 DUN Yaojun;ZHANG Changwei;LIU Rui;MA Kai;RUI Lu;ZHANG Benqing;LIN Ye;LI Shoujun(Department of Cardiovascular Surgery,Fuwai Hospital,National Center for Cardiovascular Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing,100037,P.R.China)

机构地区：[1]中国医学科学院、北京协和医学院、国家心血管病中心、阜外医院心血管外科,北京100037

出　　处：《中国胸心血管外科临床杂志》2023年第11期1571-1579,共9页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：中央高校基本科研业务费专项资金(3332021027)。

摘　　要：目的探索左冠状动脉起源于肺动脉(anomalous left coronary artery from the pulmonary artery,ALCAPA)合并左冠状动脉壁内走行患者的手术方法,分析其早期和中期临床结果。方法纳入2014年1月—2020年9月于阜外医院接受手术矫治的ALCAPA合并左冠状动脉壁内走行患儿。分析患儿的临床资料。结果共纳入10例患者,其中男8例、女2例,中位手术年龄7.5(3~46)个月。手术方式包括7例冠状动脉去顶,2例冠状动脉去顶+冠状动脉再植,1例冠状动脉去顶+左冠状动脉开口缝闭;共7例患儿同期行其他手术,包括5例二尖瓣成形术。术后2例患儿延迟关胸,全组患儿无手术死亡,无重大并发症发生。术后胸部X线片提示平均心胸比值低于术前(0.62±0.05 vs.0.67±0.06,P=0.006);术后超声提示平均左室射血分数较术前改善,但差异无统计学意义(38.7%±15.9%vs.30.0%±16.1%,P=0.066)。10例患儿出院后均得到有效随访,随访时间为13~92(46.6±25.0)个月。随访期间,患儿未出现明显症状,且无死亡、冠状动脉并发症和其他重大并发症。末次胸部X线片检查提示平均心胸比值较术后进一步下降(0.60±0.07 vs.0.62±0.05,P=0.024);末次心脏超声检查提示平均左室射血分数亦较术后进一步改善(60.1%±9.3%vs.38.7%±15.9%,P=0.002)。结论ALCAPA合并左冠状动脉壁内走行是一种罕见的畸形,根据不同的解剖类型可采用不同的手术方式进行解剖矫治,早期及中期结果满意。Objective To summarize the surgical experience of patients with anomalous left coronary artery from the pulmonary artery(ALCAPA)and intramural left coronary artery and analyze the early and mid-term clinical outcomes.Methods The infants with ALCAPA and intramural left coronary artery who underwent surgical treatment in Fuwai Hospital from January 2014 to September 2020 were retrospectively enrolled,and the clinical data of the patients were analyzed.Results A total of 10 patients were included.There were 8 males and 2 females,with a median age of 7.5(3-46)months at surgery.The surgical techniques included coronary unroofing in 7 patients,coronary unroofing with coronary reimplantation in 2 patients,and coronary unroofing with ligation of left coronary artery ostium in 1 patient.Seven infants received additional procedures,including 5 mitral valve repair.Delayed chest closure was required in 2 infants,and no operative death or major complications occurred in the whole group.Postoperative chest radiograph showed that the mean cardiothoracic ratio was lower than that before surgery(0.62±0.05 vs.0.67±0.06,P=0.006).Postoperative echocardiography indicated that the mean left ventricular ejection fraction was increased than that before surgery,but there was no statistical difference(38.7%±15.9%vs.30.0%±16.1%,P=0.066).The follow-up was available for all 10 survivors,with an average follow-up time of 13-92(46.6±25.0)months.During the follow-up period,the patients had no obvious symptoms,death,coronary complications or other major complications.The chest radiograph at last follow-up showed that the mean cardiothoracic ratio was further decreased(0.60±0.07 vs.0.62±0.05,P=0.024).The echocardiography at last follow-up showed that the mean left ventricular ejection fraction was further improved(60.1%±9.3%vs.38.7%±15.9%,P=0.002).Conclusion ALCAPA with intramural left coronary artery is a rare malformation.It can be treated with different surgical techniques with satisfactory early and mid-term outcomes.

关 键 词：左冠状动脉起源于肺动脉 冠状动脉壁内走行 冠状动脉再植 冠状动脉去顶 

分 类 号：R726.5[医药卫生—儿科]