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目的:分析解剖矫治术后的矫正型大动脉转位(ccTGA)患者的长期结果探索解剖矫治策略。方法:回顾2004年8月至2019年5月我院连续行解剖矫治手术的共120例矫正型大动脉转位患儿临床资料，通过电话随访并预约返院复查彩色超声心动图获取患者术后中长期结果。36例合并非限制性室间隔缺损/左心室流出道狭窄/异位心或右心室功能不良(8例三尖瓣下移畸形，3例右心室发育不良)接受半Mustard+Rastelli+双向Glenn手术(一个半心室矫治组)，年龄(4.6±2.2)岁，体质量(17.7±5.9)kg，中位随访时间49个月(20～84个月)；49例接受Senning+大动脉调转术(AS组，13例合并非限制性室间隔缺损，36例单纯ccTGA)，年龄(3.4±2.7)岁，体质量(17.7±11.4)kg，中位随访时间46个月(18～108个月)；24例合并非限制性室间隔缺损及左心室流出道狭窄患者接受Senning+Rastelli术(RS组)，年龄(5.7±4.3)岁，体质量(19.1±8.6)kg，中位随访时间35个月(7～84个月)；14例合并远离性室间隔缺损及左心室流出道狭窄患儿接受双动脉根部调转(DRT)+Senning术(DS组)，年龄(6.9±4.8)岁，体质量(23.0±12.9)kg，中位随访时间98个月(72～145个月)。结果:术后院内死亡6例，随访死亡2例，总体5年生存率(84.0±6.0)%，10年生存率(84.0±6.0)%。术后永久性起搏器置入6例。远期解剖三尖瓣中量及以上反流7例，其中一个半心室矫治组4例，AS组2例，RS组1例。左心室射血分数(LVEF) 一个半心室矫治组0.61±0.09，AS组0.63±0.08，RS组0.59±0.01，DS组0.65±0.07。3例出现心功能衰竭，其中一个半心室组1例(3.13%)，AS术1例(2.13%), RS术1例(4.17%)。远期再次手术2例，总体5年免于再手术率(95±11.8)%，10年免于再手术率(89.0±11.8)%。针对36例单纯ccTGA患儿，根据是否行肺动脉环缩术分为一期解剖矫治和分期矫治2个亚组，一期矫治组术后LVEF明显低于分期矫治组，(0.54±0.09)对(0.65±0.08)，n P＝0.00；一个半心室矫治组术后再手术率明显少于RS组(0对13.6%，n P＝0.03)。n 结论:对于非限制性室间隔缺损/左心室流出道狭窄/异位心或右心室功能不良患儿，一个半心室矫治能够降低心房板障梗阻率，延长右心室肺动脉外管道使用时间。单纯ccTGA患儿出现三尖瓣少量及以上反流时行肺动脉环缩术训练左心室，二期行双调转手术是理想的解剖矫治策略。合并远离型室间隔缺损/左心室流出道狭窄(LVOTO)的ccTGA患儿，Mustard或Senning+DRT是可选择一种解剖矫治术式。“,”Objective:To explore the anatomic repair strategy for congenital corrected transposition of great arteries (ccTGA).Methods:At the retrospective study, from August 2004 to May 2019, all 120 consecutive ccTGA were included and all accepted anatomic repair. There were 36 cases with with left ventricular outlet obstruction(LVOTO) and cardiac malpositon [ages(4.6±2.2) years, weight(17.7±5.9)kg] underwent the one and a half ventricle repair(hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli), 49 cases[ages(3.4±2.7) years, weight(17.7±11.4)kg] underwent double switch operation(Great artery swtich with Senning operation), 24 cases [ages(5.7±4.3) years, weight(19.1±8.6)kg] with LVOTO and ventricular sept defect(VSD) accepted the Rastelli with Senning operation, and 14 cases with LVOTO and remote VSD [ages(6.9±4.8) years, weight(23.0±12.9)kg] accepted the Double root transposition(DRT) with Senning operation. Follow up data were collected by telephone interviews and echo. The median follow-up time were 49 months varied from 20 to 84 months, 46 months varied from 18 to 108 months, 35 months varied from 7 to 84 months and 98 months varied from 72 to 145 months. Statistical analysis was performed with SPSS 19.0.Results:There were 6 in-hospital deaths and 2 follow-up deaths. The survival probability were(84.0±6.0)% and(84.0±6.0)% at 5 and 10 years after operation. The probability of freedom from re-intervention were(95.0±11.8)% and(89.0±11.8)% at 5 and 10 years after operation. All 6 patients need implant pacemaker for Ⅲ A-V block. Seven patients had moderate or more than moderate tricuspid regurgitation. The left ventricular(systemic ventricle) EF were 0.61±0.09, 0.63±0.08, 0.59±0.01 and 0.65±0.07 in one and a half ventricle repair group, double switch(AS group), Rastelli with Senning(RS group) and DRT with Senning(DS group) patients. There were 1 heart failure in one and a half ventricle repair group, 1 in AS group and 1 in RS group. For 36 pure ccTGA patients, compared with direct double switch patients these patients accepting double switch after pulmonary banding(PAB) had more EF(0.54±0.09 vs. 0.65±0.08, n P=0.00). There were significantly less patients need re-operation in one and a half ventricle repair group compared with RS group(0 vs. 13.6%, n P=0.03).n Conclusion:For ccTGA/LVOTO/cardiac malpositon, the one and a half ventricle repair was ideal strategy with significant less RV-PA conduit stenosis and re-operation. For pure ccTGA patients, second staged double switch after PAB had better long-term heart function. For ccTGA/ LVOTO/ remote VSD patients DRT with Senning was ideal strategy.