分期双调转解剖矫治术治疗左心室退化的矫正型大动脉转位的临床疗效分析

目的评价分期双调转解剖矫治术治疗先天性左心室退化矫正型大动脉转位（CCTGA）的临床疗效。方法回顾性分析2005年5月至2011年5月北京阜外心血管病医院14例左心室退化CCTGA患儿经手术治疗的临床资料，男8例，女6例；年龄2．5～72．0（34．4-24．0）个月；体重5．1～23．0（12．7±4．9）kg。术前经超声心动图、心血管造影或心导管检查确诊。主要合并畸形：三尖瓣关闭不全13例，限制型室间隔缺损10例，房间隔缺损或卵圆孔未闭7例，动脉导管未闭4例，肺动脉瓣轻度狭窄4例，Ⅲ度房室传导阻滞1例。在全身麻醉下完成一期左心室训练术，在体外循环下完成二期心房大动脉双调转术，平均间隔时间0．67～34．0（10．23±9．47）个月。结果一期左心室训练术后无住院死亡，随访结束时形态左心室舒张期末内径（mLVEDd）及形态左心室（mLV）后壁厚度均较术前显著增加（P〈0．05），室间隔位置较术前略向形态学有心室侧（mRV）移位，三尖瓣关闭不会（TR）较术前减轻，跨肺动脉环缩带压差显著增加（P〈0．05），左心室射血分数（LVEF）无显著变化；形态左心室压力与形态右心室压力比值（PmLV／PmRV）显著增大（P〈0．05）。二期心房大动脉双调转术住院死亡2例，死亡率14．3％（2／14）；死亡原因：严重心律失常、循环衰竭、猝死。术后早期并发症：肺部感染6例，房性心律失常2例，胸腔积液2例，气胸、膈肌麻痹行膈肌折叠术、气道出血、主动脉瓣轻度关闭不全、腹膜透析、体外膜肺氧合辅助、二次气管内插管各l例。生存患者均痊愈出院。术后随访12例，随访时间2～8年。随访期间死亡1例，死亡率8．33％（1／12），死亡原因：严重心律失常、循环衰竭。心功能I级8例，Ⅱ级3例。主要晚期并发症：左心功能不全3例，主动脉瓣中度关闭不全3例，二尖瓣中度关闭不全1例。结论分期双凋转解剖矫治术治疗左心室退化CCTGA早期临床疗效满意，中远期疗效尚需进一步随访观察，但术后左心功能不良和新出现的主动脉瓣关闭不全应值得关注。

Clinical Results of Left Ventricular Retraining Followed by Double Switch Operation for Congenitally CorrectedTransposition of the Great Arteries with a Deconditioned Morphologically Left Ventricle

Objective To evaluate clinical results of left ventricular retraining followed by double switch operation （DSO） for patients with congenitally corrected transposition of the great arteries （CCTGA） and a deconditioned morpho- logically left ventricle （mLV）. Methods Clinical data of 14 patients with CCTGA and a deconditioned mLV who un- derwent surgical therapy in Fu Wai Hospital from May 2005 to May 2011 were retrospectively analyzed. There were 8 male and 6 female patients with their age of 2.5-72.0 （ 34.4±24.0 ） months and body weight of 5.1-23.0 （ 12.7 ±4.9 ） kg. Preoper- ative diagnosis was confirmed by echocardiography, angiography or cardiac catheterization. Major concomitant anomalies included tricuspid regurgitation （TR） in 13 patients, restrictive ventricular septal defect in 10 patients, atrial septal defect or patent foramen ovale in 7 patients, mild pulmonary valve stenosis in 4 patients, patent ductus arteriosus in 4 patients, andthird-degree atrioventricular block in 1 patient. All the patients underwent first-stage morphologic left ventricular retraining under general anesthesia followed by second-stage atrial switch and arterial switch operations （DSO） under cardiopulmo- nary bypass with the interval of 0.67-34.0 （ 10.23± 9.47） months. Results After the first-stage morphologic left ventric- ular retraining, there was no postoperative complication or death. During follow-up, mLV end-diastolic diameter （mLVEDd） and posterior wall thickness of mLV were significantly larger than preoperative parameters （P 〈 0.05 ）. The interventricular septum moved partially towards morphologically right ventricle （mRV）. TR degree was significantly decreased, the pressure gradient across the pulmonary artery band was significantly increased （P 〈 0.05 ）, and left ventricular ejection fraction （LVEF） was not statistically different from preoperative LVEF. And mLV/mRV pressure ratio was significantly increased （P 〈 0.05）. After the second-stage DSO, 2 patients died with the in-hospital mortality of 14.3% （2/14）. The causes of death included serious arrhythmia, circulatory collapse and sudden death. Early postoperative complications included pulm- onary infection in 6 patients, atrial arrhythmias in 2 patients, pleural effusion in 2 patients, pneumothorax in 1 patient, dia- phragmatic paralysis cured by diaphragm placation in 1 patient, respiratory tract hemorrhage in 1 patient, mild aortic insuf- ficiency in 1 patient, peritoneal dialysis for 1 patient, extracorporeal membrane oxygenation for 1 patient, and tracheal intu- bation for a second time for 1 patient. All the 12 patients who were discharged alive were followed up for 2 to 8 years. One patient died during follow-up with the late mortality of 8.33% （1/12）, and the cause of death was serious arrhythmia and circulatory collapse. Eight patients were in New York Heart Association （NYHA） class I, and 3 patients were in NYHA class I]. Major late complications included left ventricular dysfimction in 3 patients, moderate aortic valve regurgitation in 3 patients, and moderate mitral valve regurgitation in 1 patient. Conclusion Short-term clinical results of left ventricular retraining followed by DSO for patients with CCTGA and a deconditioned mLV are satisfactory,and its middle- and long-term results need further follow-up. But postoperative left ventricular dysfunction and new-onset aortic valve regurgitation deserve more attention.