目的:总结左冠状动脉起源于肺动脉(ALCAPA)合并重度左心功能不全患者的外科治疗结果.方法:回顾性分析2009-02至2016-04,我院小儿外科中心采用冠状动脉再植治疗ALCAPA合并重度左心功能不全患者[左心室射血分数(LVEF) <30%] 24例,男性13例,中位年龄7.0(5.0,17.8)个月,术前中位LVEF值为21.0%(17.3%,26.5%).结果:24例患者院内死亡2例(8.3%),体外循环中位时间109 (95,128) min,主动脉阻断中位时间65 (48,87) min,呼吸机辅助中位时间94.5(48.3,165.5)h,住重症监护病房中位时间176.5(101.0,305.3)h,2例患者行体外膜肺氧合(ECMO)辅助并成功脱机,二次气管插管2例.22例患者出院时LVEF为26.0%(20%,35%)与术前比较差异无统计学意义(P>0.05).22例患者平均随访时间(15.3±14.9)个月,最长随访63个月,随访期间无再次入院、再次手术和死亡,心功能分级均为纽约心脏协会(NYHA)心功能分级Ⅰ级,最后一次随访LVEF 60%(50%,69%)较出院时显著提高(P<0.05),4例患者LVEF<50%.结论:对于ALCAPA合并重度左心功能不全的患者,外科治疗仍具有满意的近中期效果,其围术期处理强调外科、麻醉、体外循环以及术后监护室的综合努力.
Objective:To summarize the surgical treatment result in patients with anomalous left coronary artery from pulmonary artery (ALCAPA) combining severe left ventricular dysfunction.Methods:A total of 24 ALCAPA patients combining severe left ventricular dysfunction received coronary reimplantation in our hospital from 2009-02 to 2016-04 were retrospectively studied.The patients' mean age was 7.0 (5.0,17.8)months including 13 male;mean left ventricular ejection fraction (LVEF)<30% and the median pre-operative LVEF was 21.0% (17.3%,26.5%).Results:There were 2/24 (8.3%) in-hospital death,The median cardiopulmonary bypass time was 109 (95,128) min,aorta cross-clamp time 65 (48,87) min,mechanical ventilation time 94.5 (48.3,165.5) h and ICU stay time 176.5 (101.0,305.3) h;2 patients received ECMO support and weaned off successfully and 2 patients received re-intubation.In 22 survival patients,the median discharge LVEF was 26.0% (20%,35%) which was similar to pre-operative condition,P>0.05.The mean follow-up time was (15.3±14.9) months at the longest of 63 months,no re-admission,reoperation and death occurred.The patients had NYHA I and the last follow-up LVEF was 60% (50%,69%) which was increased than discharge level,P<0.05,4 patients had LVEF<50%.Conclusion:Surgical treatment had satisfactory short-and mid-term outcomes in ALCAPA patients combining severe left ventricular dysfunction;comprehensive effort should be emphasized in surgery,anesthesia,cardiopulmonary bypass and ICU management at peri-operative period.