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目的 分析左冠状动脉异常起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)行左冠状动脉移植术后的疗效及中期随访结果,并探讨是否需要同期处理反流的二尖瓣.方法 2005年3月至2015年3月广东省心血管病研究所心外科共52例ALCAPA患者行左冠状动脉移植术,其中男20例、女32例,中位年龄10(2～ 193)个月,平均体质量(9.7±7.8)kg.按同期有无行二尖瓣成形术分为二尖瓣成形组(MVP组)及非二尖瓣成形组(Non-MVP组).其中Non-MVP组35例,男14例、女21例,年龄(35.4±42.8)个月;MVP组17例,男6例、女11例,年龄(13.5±11.0)个月.为便于量化统计,二尖瓣反流按无、局限性、轻度、轻-中度、中度、中-重度及重度分别定义为0.0、1.0、2.0、2.5、3.0、3.5、4.0.结果 术后左室短轴缩短率(LVFS)及左室舒张期末内径(LVEDD)均较术前明显改善[32.1％±10.1％ vs.28.6％±9.6％,P=0.023;(30.5±5.7)mm vs.(38.4±5.6)mm,P＜0.001],术后二尖瓣反流程度亦较术前明显减轻(2.4±1.2 vs.2.9±1.2,P=0.001).术后早期死亡4例,死亡率7.7％ (4/52).中位随访时间21.0 (1.5～ 111.0)个月,3例(5.8％)失访,1例(1.9％)接受再次手术,随访期间无死亡.末次随访LVFS较术后明显改善(38.0％±6.0％ vs.32.1％±10.1％,P=0.001);末次随访二尖瓣反流程度与术后差异无统计学意义(2.3±1.2 vs.2.4±1.2,P=0.541),但有减轻趋势.两组体外循环时间、主动脉阻断时间、机械通气时间及住院时间差异并无统计学意义.结论 采用左冠状动脉移植术恢复双冠状动脉供血,可显著改善ALCAPA患者左心室功能,是否需要同期处理反流的二尖瓣仍然存在争议.对于ALCAPA合并中-重度及重度二尖瓣反流的患者,同期行二尖瓣成形术有助于二尖瓣功能的早期恢复.“,”Objective To analyze the clinical efficacy and mid-term outcomes of reimplantation of anomalous origin of left coronary artery from the pulmonary artery (ALCAPA),and to evaluate whether concomitant management of mitral regurgitation (MR) during ALCAPA repair is needed.Methods Between March 2005 and March 2015,52 consecutive patients (20 males and 32 females with a median age of 10 months ranging 2-193 months) underwent reimplantation of ALCAPA at Department of Cardiac Surgery,Guangdong Cardiovascular Institute.There were 14 males and 21 females with a mean age of 35.4±42.8 months not receiving mitral valvuloplasty (a Non-MVP group),and 6 males and 11 females with a mean age of 13.5±11.0 months receiving mitral valvuloplasty (a MVP group).In order to facilitate the analysis,degree of MR was graded by number:0.0=none,1.0=trivial,2.0=mild,2.5=mild-moderate,3.0=moderate,3.5=moderate-severe and 4.0=severe.Results The left ventricular fractional shortening (LVFS) and left ventricular end diastolic diameter (LVEDD) demonstrated significant improvement between preoperation and discharge (28.6％±9.6％ vs.32.1％±10.1％,P=0.023;38.4±5.6 mm vs.30.5±5.7 mm,P＜0.001),and there was also significant improvement in the mean MR grade between preoperation and discharge (2.9± 1.2,vs.2.4±1.2,P=0.001).There were 4 in-hospital deaths (7.7％).The median follow-up was 21.0 months (ranging 1.5-111.0 months).Three patients (5.8％) were lost to follow-up,1 patient required reoperation for mitral valve replacement and there was no death during follow-up.Significant improvement was seen in LVFS between discharge and final follow-up (32.1％±10.1％ vs.38.0％±6.0％,P=0.001);however,there was no significant difference in the degree of MR between discharge and final follow-up (2.4±1.2 vs.2.3±1.2,P=0.541).There was no significant difference in cardiopulmonary bypass time,aortic cross-clamping time,mechanical ventilation time or hospital stay between the two groups.Conclusion Creation of a dual coronary system with reimplantation of the left coronary artery results in complete recovery of left ventricular function.However,concomitant management of MR during ALCAPA repair remains controversial.Concomitant mitral valve repair for ALCAPA patients with moderatesevere and severe MR is helpful to early function recovery of mitral valve.