左冠状动脉起源于肺动脉合并重度左心功能不全的外科治疗

Surgical Treatment in Patients With Anomalous Left Coronary Artery From Pulmonary Artery Combining Severe Left Ventricular Dysfunction

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, re- operation 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.