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儿童左冠状动脉起源于肺动脉的术前评估及术后随访

Preoperative evaluation of children with anomalous origin of left coronary artery from the pulmonary artery and the follow-up after surgery

摘要:

目的 回顾性总结左冠状动脉起源于肺动脉(ALCAPA) 50例患儿的临床资料,探讨采用临床常用指标评价心肌存活程度的可能性和价值,并寻找影响手术和术后恢复的危险因素.方法 选取1999年4月至2013年3月收治的ALCAPA患儿50例.男29例,女21例;年龄4个月~18岁,平均(4.49±4.30)岁.术前评估基于常规临床检查;术后随访依据门诊复查结果及电话随访.对15例行核素心肌显像检查患儿行心肌存活程度分级.0:心肌正常;1:心肌存活;2:心肌大部分存活;3:心肌梗死.同时根据临床指标进行综合评分并分析心肌存活程度与临床指标的相关性.临床指标包括ECG异常Q波、左心室射血分数(LVEF)、左心室舒张末径(LVED)、侧支生成情况、胸部X线片心胸比值(CTR)、二尖瓣返流(MR)、有无室壁瘤或其他术前严重情况.临床综合评分最高为10分,最低为0分.分析评价综合评分和临床常用单项指标对心肌梗死的预测价值.在此基础上,将所有患儿按临床综合评分分组,比较其术前、围术期及术后恢复情况.结果 心肌存活程度分级与临床综合评分(r =0.936,P<0.001)、LVEF(r=0.783,P<0.001)、CTR(r=0.770,P<0.002)、异常Q波(r =0.667,P<0.01)、LVED(r =0.637,P<0.02)呈显著正相关,与年龄、MR和侧支形成情况(r=-0.206、-0.268、-0.342,P均>0.05)无显著相关性.心肌存活程度分级0~1级所对应的综合评分为0~3分;2级对应综合评分3~5分;3级对应综合评分5分以上.评分越高、心肌梗死程度越重、病情也越重.临床综合评分>3分和LVEF< 50%较心电图异常Q波、侧支生成不良、CRT> 0.65、MR中重度对心肌梗死预测价值高.所有患儿分2组(临床综合评分≤3分为A组,评分>3分为B组)比较发现,B组小年龄、心脏增大、严重心功能不全患儿较A组多.B组行冠状动脉移植术比例高于A组,行肺动脉内隧道术的比例低于A组.主动脉阻断时间和体外循环时间2组比较差异无统计学意义.呼吸机辅助和ICU滞留时间B组均明显长于A组.47例患儿行手术治疗,无手术早期死亡,1例临床综合评分9分的患儿术前死亡.术后随访38例(80.9%,38/47例),随访期限(1 ~168)个月.晚期死亡1例.所有患儿LVED均较术前缩小,仍有轻度MR 19例,残余肺动脉轻度压差3例.16例术前LVEF< 50%,14例恢复正常,2例恢复中.结论 ALCAPA患儿的存活心肌对手术和预后有重要意义,心肌存活状况与临床指标密切相关.LVEF< 50%和临床综合评分>3分对心肌梗死预测价值高.术前年龄小、心功能低下、临床综合评分高的患儿术后早期风险大,但手术建立起有效的双冠状动脉循环后,中远期心功能恢复满意.

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abstracts:

Objective To retrospectively study 50 children with anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA) and find the simple and practical indexes that may predict myocardial viability and the possible risk factors that may affect the choice of operation and the recovery after surgery.Methods A retrospective study was performed in 50 children with ALCAPA [29 male,21 female,aged from 4 months to 18 years,average (4.49 ±4.30) years] between Apr.1999 and Mar.2013.The preoperative examination included electrocardiogram (ECG),echocardiography,chest X-ray,mutislice spiral computerized tomography (MSCT),angiocardiography et al.Follow-up data were obtained by reviewing the records made in out-patient and recent telephone call.According to myocardial 18F-FDG imaging,the extent of myocardial viability of 15 patients with ALCAPA was classified into 4 grades.0 normal myocardial; 1 viable myocardial ;2 partial viable myocardial;3 myocardial infarction.Meanwhile,the global clinical scores were calculated and divided into 10 levels according to the deviations of clinical manifestations which included abnormal Q waves,left ventricular ejection fraction (LVEF),left ventricular end-diastolic dimension (LVED),cardiothoracic ratio(CTR),intercoronary collaterals(ICC),mitral regurgitation(MR) and aneurysm.The relationship of the extent of myocardial viability and clinical features were studied.All patients with ALCAPA were classified into groups by the global clinical scores and the preoperative,perioperative and post operative clinical manifestations were compared among groups.Results The extent of myocardial viability was related well to the global clinical scores (r =0.936,P <0.001),LVEF(r =0.783,P < 0.001),CTR (r =0.770,P < 0.002),abnormal Q waves (r =0.667,P < 0.01) and LVED (r =0.637,P < 0.02),but was not related to age,MR and ICC (r =-0.206,-0.268,-0.342,all P > 0.05).The results showed that grade 0-1 equaled scores 0-3,grade 2 equaled scores 3-5,grade 3 equaled scores > 5.Chil-dren with less viable myocardium had more severe clinical symptoms.Among abnormal Q waves,LVEF < 50%,CTR >0.65,ICC dysplasia,median to severe MR and the global clinical scores > 3,the global clinical scores > 3 and LVEF <50% showed a good predict of myocardial infarction.The preoperative,perioperative and post operative clinical manifestations were compared between 2 groups divided by the global clinical scores (group A,scores ≤ 3 and group B,scores >3) in all patients.More infants and toddlers,abnormal Q waves,ICC dysplasia,RCA/AO <0.2,larger CTR and lower LVEF were found in group B than those in group A.The time of cross-clamp and CPB was not different between the 2 groups,but the postoperative ventilation time and postoperative ICU stay were longer in group B than in group A.Fortyseven patients underwent operation and there were no operative deaths.One infant with scores 9 died while waiting heart transplantation.Follow-ups from 1 to 168 months were conducted in 38 patients(80.9%,38/47 cases) and 1 patient with ligation of the left coronary artery (LCA) had a sudden death after 8 months of surgery.All patients had gotten smaller LVED after surgery.Nineteen patients had mild MR and 3 patients had mild supravalvular pulmonary stenosis in following-up.Of the 16 patients with LVEF < 50%,14 had a recovery of LVEF,2 patients still had LVEF < 50%.Conclusions In children with ALCAPA,the extent of myocardial viability evaluated by myocardial 18 F-FDG imaging is related closely to the preoperative clinical manifestations.The global clinical scores > 3 and LVEF < 50% have a good predict of myocardial infarction.Even in young children with severely depressed left ventricular function,higher global clinical scores and more myocardial infarction,median and long-term follow-ups showed satisfactory recovery of cardiac function after successful restoration of a dual coronary arterial system.

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