小儿左冠状动脉异常起源于肺动脉(ALCAPA)的手术治疗

Surgery treatment of anomalous origin of the left coronary artery from pulmonary artery(ALCAPA) in children

目的总结小儿左冠状动脉异常起源于肺动脉(anomalous origin of the left coronary artery from pulmonaryartery,ALCAPA)的手术治疗经验。方法回顾性分析2005年5月至2011年12月在我中心行手术治疗的9例小儿ALCAPA的病例资料,男性4例,女性5例,平均年龄3.1±0.8岁(2个月至13岁)。其中3例采用延长左冠状动脉后再植入术,6例采用左冠状动脉直接植入术。1例患儿合并重度二尖瓣关闭不全伴返流,同期行二尖瓣成形术,术后7个月因二尖瓣重度返流伴急性左心功能衰竭行二尖瓣机械瓣置换术。结果全组病例无住院和随访死亡。平均体外循环时间(137.5±35.0)min,平均主动脉阻断时间(67±21)min,平均机械通气时间(70.6±52.6)h,平均监护室滞留时间(9.2±6.0)天。1例术后左心功能衰竭无法脱离体外循环,经体外膜肺氧合支持治疗,辅助18h撤机。随访6～40个月,平均(30±8)个月,冠状动脉血流通畅,左心室射血分数从术前40%±8%增加至术后65%±10%(P<0.05),左心室缩短分数从术前23%±7%增加至术后40%±6%(P<0.01),心功能均恢复至NYHAⅠ级。结论 ALCAPA应尽早诊断,尽早手术矫治,冠状动脉再植入术是首选术式。

Objective To summarize the experience in surgical correction of anomalous origin of the left coronary artery from pulmonary artery （ALCAPA） in children. Methods From May,2005 to Dec. ,2011, 9 patients with anomalous origin of the left coronary artery from pulmonary artery underwent surgery,including 4 boys and 5 girls,aged from 2 months to 13 years . In total,6 patients underwent direct aortic reimplantation of ALCAPA with a button of pulmonary artery, 3 patients underwent aortic reimplantation of ALCAPA after lengthened by pulmonary artery wall and pericardium. One patient received replacement of mitral valve 7 months after first surgery （including piasty of mitral valve） due to severe MI and MR. One patient received extracorporeal membrance oxygenation support for 18 hours due to severe acute left hear failure postoperatively. Definite diagnosis was made by chest X-ray, ECG, echocardiogram and cardiac catheter examination in all patients. Results No hospital death and later deaths during follow-up. The mean cardiopulmonary bypass time was （137.5 ±35.0） minutes and the mean aortic clamp time was （67± 21） minutes. The mean mechanicalventilation time was （70.6±52.6） hours . The mean intensive care stay time was （9.2 ± 6.0） days. The median length of follow-up was （30± 8） months. All the followed-up patients presented no myocardial ischemia or infarction. Cardiac function was rehabilitated to New York Heart Association function class I ,mean LVEF 65% ± 10% （vs. 40% ± 8% before the procedure,P〈0.05） ,and mean LVFS 40% ± 6% （vs. 23% ± 7% before the procedure,P〈0.01）. All patientsr MR were mild except for MV replaced patient. Conclusions Early diagnosis and surgery should be done for ALCAPA patients. The aortic implantation of ALCAPA may be the first choice.