伴左心室流出道梗阻的完全性大动脉转位行动脉调转术后左心室流出道及主动脉瓣功能随访  

Outcome of Let Ventricular Outlet Tract and Aortic Valve Function at er Arterial Switch Operation for Patients with Transposition of the Great Arteries and Left Ventricular Outlet Tract Obstruction

作　　者：畅怡[1] 高华炜[1] 闫军[1] 花中东[1] 张浩[1] 杨克明[1] 楚军民[1] 李守军[1] 

机构地区：[1]中国医学科学院北京协和医学院国家心血管病中心阜外心血管病医院心血管疾病国家重点实验室小儿外科中心,北京100037

出　　处：《中国胸心血管外科临床杂志》2015年第2期123-127,共5页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：协和创新团队基金资助项目(2011-XH7)~~

摘　　要：目的探讨伴左心室流出道梗阻的完全性大动脉转位患者行动脉调转术后,左心室流出道梗阻的改善情况及主动脉瓣功能情况。方法 2002~2013年共549例患儿于阜外心血管病医院行动脉调转术,其中42例患者合并左心室流出道梗阻,其中男31例、女11例,中位月龄12个月（7 d至96个月）;中位体重6.5（3.5~26.0）kg,外周经皮血氧饱和度52%~85%;左心室流出道病变类型包括肺动脉瓣异常,瓣下隔膜,隧道样狭窄,肌性狭窄,附属瓣膜组织及复合病变。术中根据病变类型采取不同方法：瓣交界粘连行交界切开,瓣下隔膜予以切除,单纯肌性狭窄则切除肥厚肌束或部分室间隔,环形或隧道样狭窄则切除纤维组织和肥厚肌肉,副瓣样组织或无功能腱索,予以切除,通过室间隔缺损跨越至左心室的腱索,切下重植。结果平均体外循环时间147~344（193.5±73.1）min,主动脉阻断时间139（109~305）min,呼吸机使用时间36（3~960）h,住ICU时间5（1~48）d。体外膜式氧合（ECMO）辅助3例,均成功撤除。早期死亡2例,1例为多器官功能衰竭,1例为严重感染。随访期间死亡1例,原因不明,失访3例,接受随访患者36例,随访时间24（3~116）个月;再发左心室流出道梗阻1例,为瓣下局限增厚纤维组织所致,新主动脉瓣轻度狭窄1例,新主动脉瓣少量反流11例,中量反流2例;随访时中位左心室-主动脉压差4（2~49）mm Hg,较术前[37.2（12.1~70.6）mm Hg]有明显改善（Z=-5.153）。1年时心脏事件免除率为91%±5%,5年时为78%±8%。结论对于合并左心室流出道梗阻的完全性大动脉转位,需结合解剖情况与压差评估梗阻严重程度,指征把握恰当,行动脉调转术可获得满意的中远期效果。Objective To assess the function of left ventricular outlet tract and aortic valve after arterial switch operation（ASO） for patients with transposition of the great arteries（TGA） and left ventricular outlet tract obstruction（LVOTO）. Methods From 2002 to 2013, 549 pediatric TGA patients received ASO in Fu Wai Hospital. Among them, 42 patients had LVOTO, including 31 males and 11 females with their median age of 12 months（range, 7 days to 96 months）, median body weight of 6.5（3.5-26.0） kg and percutaneous oxygen saturation of 52%-85%. LVOTO anomalies included pulmonary valve stenosis, subaortic membrane, tunnel-like subaortic stenosis, muscular subaortic stenosis, subvalvular apparatus and combined anomalies. Different surgical procedures were performed according to respective anomalies. Echocardiographic characteristics, intraoperative findings, surgical methods, early and follow-up results were summarized. Results Cardiopulmonary bypass time was 147-344（193.5±73.1） minutes, mean aortic cross-clamping time was 139（109-305） minutes,mean mechanical ventilation time was 36（3-960） hours,and mean length of ICU stay was 5（1-48） days. Three patients received and later successfully weaned from extracorporeal membrane oxygenation. Two patients died postoperatively including 1 patient with multiple organ dysfunction syndrome and another patient with severe infection. One patient died during follow-up for unknown reason, and 3 patients were lost during followup. Thirty-six patients were followed up for 24（3-116） months. During follow-up, there were 1 patient with LVOTO recurrence, 1 patient with new-onset mild aortic valve stenosis, 11 patients with new-onset mild aortic regurgitation（AR）, and 2 patients with new-onset moderate AR. Median systolic left ventricular-aortic pressure gradient [4（2-49）mm Hg] was significantly lower than preoperative value [37.2（12.1-70.6） mm Hg]（Z=-5.153）. Cardiac event-free rate was 91%±5% at 1 year and 78%±8% at 5 years after discharge.

关 键 词：完全性大动脉转位 左心室流出道梗阻 动脉调转术 

分 类 号：R726.5[医药卫生—儿科]