左冠状动脉起源于右肺动脉合并冠状动脉壁内走行的外科治疗  

Surgical intervention for anomalous origin of left coronary artery from right pulmonary artery with intramural aortic course

作　　者：张辉[1] 程沛[2] 金戈[2] 罗毅[1] 

机构地区：[1]首都儿科研究所心脏外科,北京100020 [2]首都医科大学附属北京安贞医院小儿心脏科,100029

出　　处：《中国医药》2016年第2期169-172,共4页China Medicine

摘　　要：目的总结左冠状动脉起源于右肺动脉合并冠状动脉壁内走行的外科治疗经验。方法选择2007年10月至2014年8月于首都医科大学附属北京安贞医院接受手术治疗的左冠状动脉起源于右肺动脉合并冠状动脉主动脉壁内走行患儿10例，其中6例合并二尖瓣反流（轻度2例，中重度4例），5例合并左心室室壁瘤。术前左心室射血分数（LVEF）34％-64％；左心室缩短分数（LVFS）10％～33％。患儿均采用胸正中切口全身麻醉体外循环下重建双冠状动脉法，包括直接冠状动脉移植或冠状动脉去顶。4例中重度二尖瓣反流患儿同期行二尖瓣交界环缩成形术，2例轻度二尖瓣反流未处理。5例室壁瘤患儿中2例行室壁瘤切除术，另外2例行室壁瘤折叠缝缩术，1例未处理。结果10例患儿中2例死亡，死亡原因均为低心排血量综合征。8例存活患儿随访13～72个月，左冠状动脉血流通畅，LVEF53％～72％，LVFS27％-40％，较术前明显改善。4例行二尖瓣交界环缩成形患儿中2例反流消失，2例轻度反流。另2例二尖瓣未处理患者较术前无明显改变。4例室壁瘤手术患儿，3例室壁运动异常范围明显缩小，1例术后死亡。患儿均未行二次手术。结论单纯左冠状动脉起源于右肺动脉是一种少见畸形，常合并冠状动脉主动脉壁内走行。根据不同解剖类型采用不同冠状动脉移植方式能够取得满意的冠状动脉移植效果。远期疗效仍需要进一步随访。Objective To explore the experience of surgical treatment for anomalous origin of left coronary artery from the right pulmonary artery with intramural aortic course. Methods Totally 10 patients with anomalous origin of left coronary artery from right pulmonary artery with intramural aortic course from October 2007 to August 2014 underwent surgery; among them, 6 cases were complicated with mitral regurgitation （MI） （2 cases of mild MI and 4 cases of moderate-severe MI）, 5 cases were complicated with left ventricular aneurysm. Before operation, the left ventricular ejection fraction （LVEF） was 34%-64% , the left veutrieular fractional shortening （LVFS） was 10%-33%. All patients were reconstructed with double-coronary artery, including direct coronary artery graft and coronary unroofing technique, under general anesthesia with middle thoracic incision. In 6 eases with MI, 4 cases with severe MI underwent annuloplasty simultaneously; in patients with ventrieular aneurysm, 2 eases underwent left ventricular aneurysm excision and 2 eases underwent folding closure simultaneously. Results Two eases were died from low cardiac output syndrome after operation. Eight eases survived and were followed-up for 13-72 months, showing good blood flow of left coronary artery and improved cardiac function, with LVEF of 53%-72% and LVFS of 27%-40%. In 4 cases undergoing annuloplasty, MI was disappeared in 2 cases and turned to mild in 2 eases; in 4 cases undergoing ventrieular aneurysm surgery, the ventricular wall motion abnormalities was significantly narrowed down in 3 patients after ventrieular aneurysm repaired. No patient need reoperation. Conclusions Anomalous origin of left coronary artery from the right pulmonary artery is rare, usually combined with intramural aortic course. According to the anatomical type, different eoronary transplantation methods can obtain satisfactory effect, but the long-term effect still need further follow-up.

关 键 词：左冠状动脉起源异常 肺动脉 壁内走行 外科治疗 

分 类 号：R543.3[医药卫生—心血管疾病]