左冠状动脉起源于肺动脉小儿患者的外科治疗

Surgical Treatment of Children with Anomalous Left Coronary Artery from the Pulmonary Artery

目的总结我院小儿左冠状动脉起源于肺动脉（ALCAPA）的外科治疗结果。方法回顾性分析2004年4月至2015年2月我院小儿外科中心行冠状动脉再植术治疗小儿ALCAPA患者56例的临床资料,其中男35例、女21例,平均年龄25.5（7.3~60.0）个月,其中19例患者年龄小于1岁,平均体重11.8（7.8~19.8）kg。结果住院死亡1例,体外循环时间（131.8±61.2）min,主动脉阻断时间（83.4±32.1）min,呼吸机辅助时间和住ICU时间分别为12.5（6.5~43.8）h和49.0（1.0~116.0）h。3例患者行体外膜肺氧合（ECMO）辅助并成功脱机,出院时左心室射血分数（LVEF,63.4%±15.8%vs.50.6%±18.7%）和左心室舒张期末内径[LVEDD,（36.4±32.5）mm vs.（42.3±7.4）mm]均较术前显著改善,15例行二尖瓣成形术的患者术后二尖瓣反流（MR）情况为中度2例,轻度8例,微量2例,其余3例无反流。其他41例未行二尖瓣成形术的患者MR程度保持不变或改善。平均随访时间（45.4±23.6）个月。随访期间1例死于非心源性原因,无再次手术及入院,所有患者心功能分级（NYHA）Ⅰ级或Ⅱ级,最后一次随访时患者LVEF（62.8%±5.0%）较出院时显著提高,MR中度6例,轻度24例,微量4例,21例患者无反流。结论冠状动脉再植术对小儿左冠状动脉起源于肺动脉患者近中期效果良好。对于合并二尖瓣大量反流并有乳头肌缺血表现的患者同期行二尖瓣成形术效果良好。

Objective To review the experience of the surgical treatment of child patients with anomalous left coronary artery from the pulmonary artery（ALCAPA）. Methods We retrospectively analyzed the clinical data of 56 children patients with ALCAPA underwent coronary re-implantation in our hospital from April 2004 through February2015. There were 35 males and 21 females at mean age of 25.5（7.3-60.0） months. Nineteen patients（33.9%） were less than 1 year of age. The mean weight was 11.8（7.8-19.8） kg. Results There was one death in-hospital. The mean cardiopulmonary bypass time and cross-clamp time was 131.8±61.2 min and 83.4±32.1min, respectively. The mean mechanical ventilation time and intensive care unit time was 12.5（6.5-43.8） h and 49.0（21.0-116.0） h, respectively. Three patients underwent extracorporeal membrane oxygenation（ECMO） support and weaned off successfully. The mean postoperative left ventricular ejection fraction（LVEF, 63.4%±15.8% vs. 50.6%±18.7%） and left ventricular end diastolic diameter（LVEDD, 36.4±32.5 mm vs. 42.3±7.4 mm） significantly improved compared postoperative（P〈0.05）. The mitral regurgitation（MR） distribution in the 15 patients underwent mitral valve repair was： moderate in 2 patients, mild in 8patients, trivial in 2 patients and none in 3 patients. The MR in the other 41 patients improved or did not change. The survivors completed the follow-up for a mean time of 45.4±23.6 months. During the follow-up period, one patient died due to noncardiac reason. No patient required reoperation or readmission. All the patients survived with New York Heart Association heart function class Ⅰ or class Ⅱ. At the latest echocardiography, the mean LVEF（62.8%±5.0%） significantly improved compared with the LVEF of discharge. The MR distribution was moderate in 6 patients, mild in 24 patients,trivial in 4 patients and none in 21 patients. Conclusion The coronary re-implantation has a satisfactory mid-term result for patients with ALCAPA. Mitral valve repair is recommended for the patients with severe regurgitation and evident ischemic lesions of the papillary muscles.