左冠状动脉异常起源于肺动脉围术期处理

Perioperative Management of Anomalous Origin of the Left Coronary Artery from Pulmonary Artery

目的总结16例左冠状动脉异常起源于肺动脉围术期处理经验。方法回顾性分析2005年1月至2013年12月复旦大学附属儿科医院心血管中心收治16例左冠状动脉异常起源于肺动脉患儿的临床资料,其中男6例、女10例,手术年龄为2个月至13岁,平均（23.25±0.63）个月,体重5~30（19.77±0.75）kg。全组均行冠状动脉移植术,并综合管理左心室射血分数、心率、血压、动脉血气分析、尿量、血乳酸、中心静脉压等。结果术后出现低心排血量综合征9例,其中难治性低心排血量综合征2例;心律失常1例;全组共1例因难治性低心排血量综合征合并严重心律失常死亡,死亡率为6.25%;体外循环时间为85~260（135.61±35.01）min,主动脉阻断时间为40~97（57.32±16.02）min,机械通气时间34~187（106.34±41.62）h;住CICU时间2~21（8.13±5.02）d,住院时间12~51（22.14±5.00）d,术后左心室射血分数和左心室缩短分数较术前提高（P〈0.05）。结论左冠状动脉异常起源于肺动脉围术期系统性处理,术前心功能维护,积极预防各种并发症及新技术应用是手术成功的保障。

Objective To summarize perioperative management experience of 16 patients undergoing surgical correction of anomalous origin of the left coronary artery from pulmonary artery（ALCAPA）. Methods We performed a retrospective analysis of 16 patients who received surgical correction of ALCAPA between January 2005 and December 2013 in Cardiovascular Center of Children＇s Hospital of Fudan University. There were 6 males and 10 females with their age ranging from 2 months to 13 years（mean age of 23.25±0.63 months） and body weight ranging from 5 to 30 kg（mean body weight of 19.77±0.75 kg）. All the patients underwent direct implantation of the anomalous coronary artery into the ascending aorta,and received comprehensive management regarding left ventricular ejection fraction（LVEF）, heart rate, blood pressure, arterial blood gas, urinary volume, blood lactic acid and central venous pressure. Results Postoperative complications were low cardiac output syndrome（LCOS） in 9 patients（including intractable LCOS in 2 patients） and arrhythmia in 1 patient. One patient with intractable LCOS and arrhythmia died postoperatively with the mortality of 6.25%. Cardiopulmonary bypass time was 85-260（135.61±35.01） minutes, aortic cross-clamping time was 40-97（57.32±16.02） minutes, mechanical ventilation time was 34-187（106.34±41.62） hours, length of CICU stay was 2-21（8.13±5.02） days, and hospital stay was 12-51（22.14±5.00） days. Postoperative LVEF and left ventricular fractional shortening were significantly higher than preoperative values（P0.05）. Conclusion Key points for successful surgical correction of ALCAPA include meticulous perioperative management, preoperative heart function improvement, prevention of postoperative complications and use of new techniques.