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目的总结完全性大动脉转位(TGA)矫治手术的围术期处理措施及影响预后的因素。方法2004年1月-2008年12月行TGA外科矫治术患儿230例(年龄<7岁)。根据临床诊断分组:Ⅰ组为室间隔完整的TGA(TGA/IVS,n=73),Ⅱ组为伴室间隔缺损(VSD)的TGA(TGA/VSD,n=118),Ⅲ组为伴左室流出道狭窄的TGA(TGA/LVOTO,n=39)。Ⅰ组和Ⅱ组患儿行大动脉调转术(ASO);Ⅲ组患儿行Rastelli手术、改良REV手术和改良Nikaidoh手术。结果230例患儿中位数年龄为0.3岁(1d～7岁);中位数体质量为5.0kg(2.4～23.0kg)。Ⅲ组体外循环和主动脉阻断时间中位数分别为279min(100～501min)和191min(80～281min),为3组中最长。3组共死亡22例(9.6%)。其中Ⅰ组死亡10例(13.7%),2例术前有严重代谢性酸中毒,未行球囊房间隔扩张术,急诊ASO手术后死于低心排出量;3例发生急性左心衰竭死亡;余5例死亡与伴心血管畸形有关。Ⅱ组死亡9例(7.6%),3例突发心律失常死亡;3例二次气管插管后并肺部感染死亡;1例并主动脉弓缩窄,同期手术矫治,手术后发生脑部并发症死亡;2例左心功能训练手术后肺功能较差,急诊行ASO手术后死亡。Ⅲ组死亡3例(7.5%),其年龄均小于本组中位数年龄,死亡原因为手术后长时间低心排出量。16例伴冠状动脉异常,其中8例术后死亡。结论TGA/IVS患儿手术前球囊房间隔扩张处理并严重酸中毒患儿,明显影响手术结果,体外循环后注意左心功能的维护。TGA/VSD患儿突发心律失常和术后肺内感染是主要死亡原因。TGA/LVOTO患儿手术时间长,低龄患儿手术死亡危险增加。TGA患儿伴冠状动脉异常病死率增加。
Objective To summarize the perioperative management and prognostic factors of complete aortic transposition (TGA). Methods From January 2004 to December 2008, 230 patients (aged <7 years) underwent TGA surgical correction. According to the clinical diagnosis, group Ⅰ was TGA (TGA / IVS, n = 73), group TGA (TGA / VSD, n = 118) with ventricular septal defect (VSD) Room outflow tract narrow TGA (TGA / LVOTO, n = 39). Group Ⅰ and group Ⅱ children underwent aortic valve surgery (ASO); group Ⅲ children underwent Rastelli surgery, modified REV surgery and modified Nikaidoh surgery. Results The median age of 230 infants was 0.3 years (ranged from 1 d to 7 years). The median body mass was 5.0 kg (2.4-23.0 kg). The median time of cardiopulmonary bypass and aortic blockage in group Ⅲ were 279min (100 ~ 501min) and 191min (80 ~ 281min) respectively, which was the longest among the three groups. Totally 22 patients died in 3 groups (9.6%). Among them, 10 patients (13.7%) died in group Ⅰ, 2 patients had severe metabolic acidosis preoperatively, no balloon atrial septal expansion, and died of low cardiac output after emergency ASO operation; 3 patients died of acute left heart failure ; 5 cases of death associated with cardiovascular malformations. In group Ⅱ, 9 patients died (7.6%), 3 patients died of sudden arrhythmia, 3 died of pulmonary infection after secondary intubation, and 1 patient had aortic arch constriction. Surgery was performed in the same period and brain complications occurred after operation Died; 2 cases of left heart function training lung function worse, emergency ASO died after surgery. Three patients died in group Ⅲ (7.5%). Their ages were less than the median age of the patients. The cause of death was low cardiac output after long-term operation. 16 patients with coronary artery abnormalities, of which 8 patients died after surgery. Conclusion TGA / IVS infants with balloon dilation before operation and severe acidosis in children with TGA / IVS have a significant effect on the surgical outcome. They should pay attention to the maintenance of left ventricular function after cardiopulmonary bypass. TGA / VSD children with arrhythmia and postoperative pulmonary infection is the leading cause of death. TGA / LVOTO children with a long operation, young children with increased risk of surgical death. TGA children with coronary artery abnormalities increased mortality.