完全性大动脉转位合并室间隔缺损及左心室流出道梗阻的手术治疗

Operative treatment of complete transposition of great arteries with ventricular septal defect and left ventricular outflow tract obstruction

目的总结完全性大动脉转位合并室间隔缺损（VSD）及左心室流出道梗阻（TGA-VSD-LVOTO）的外科治疗效果。方法自2012年10月至2015年3月连续收治9例TGA-VSD-LVOTO患者,男5例,女4例,年龄7个月至5岁,多普勒测左心室流出道或肺动脉瓣流速（4.38±0.19）m/s,肺动脉Nakata指数（209±27）cm^2/m^2,9例患者中VSD距离主动脉瓣约5-10 cm 5例,在主动脉瓣下合并主动脉骑跨4例。其中VSD远离主动脉瓣的5例患者和VSD在主动脉瓣下的1例患者采用双根部调转（DRT）治疗;其中3例VSD在主动脉瓣下合并主动脉骑跨患者采用改良REV手术治疗。结果 1例患者术后4 h出现低心排出量综合征,应用体外膜肺氧合（ECMO）失败,多器官衰竭死亡,其余患者均痊愈出院,术后随访3-32个月,8例患者均心功能1-2级（NYHA）,左心室射血分数（LVEF）（64±2.4）%,肺动脉瓣少量反流6例,中量反流2例,主动脉瓣未见反流5例,微量反流3例,血氧饱和度（Sa O2）（99±0.5）%。结论根据VSD位置选用DRT术和改良REV手术是治疗TGA-VSD-LVOTO的有效方法。

Objective To summarize the operative therapeutic effects of complete transposition of great arteries with ventricular septal defects（ VSD） and left ventricular outflow tract obstruction（ TGA-VSD-LVO-TO）.Methods From October 2012 to March 2015,we treated 9 patients with TGA-VSD-LVO-TO,5 male and 4 female,age ranged from 7 months to 5 years old,the velocity of left ventricular outflow or pulmonary valve showed by Doppler was（ 4. 38 ± 0. 19） m / s,Nakata index was（ 209 ± 27） cm^2/ m^2. 5 of these 9 patients were 5-10 cm form VSD to aortic valve and other 4 patients＇ VSD located below the aortic valve. 6 patients were carried out double root translocation（ DRT） operation,including 1 with VSD below aortic valve and 5 with VSD far away from aortic valve.The other 3 patients with VSD below aortic valve were treated by modified REV procedure. Results One patient developed low cardiac output syndrome 4 hours after the operation and was applied extracorporeal membrane oxygenation（ ECMO）,but died of multiple organ failure. Other 8 patients all recovered and discharged from hospital. During the 3-32 months＇ follow-up,8 patients all showed NYHA Class Ⅰ or Ⅱ,left ventricular ejection fractions（ LVEF） was（ 64 ± 2. 4） %; 6 of them had mild pulmonary regurgitation,2 were moderate pulmonary regurgitation; 5 of them had no aortic regurgitation,3 were micro aortic regurgitation,Sa O2was（ 99 ± 0. 5） %.Conclusion DRT and REV are effective treatments in management of complete transposition of great arteries with left ventricular outflow tract obstruction according to location of VSD.