先天性矫正型大动脉转位解剖纠治的外科策略及十年随访的回顾性队列研究

Anatomic correction for congenitally corrected transposition of the great arteries and 10-year follow-up: A retrospective cohort study

目的探索先天性矫正型大动脉转位(ccTGA)个体化解剖纠治的外科策略及效果。方法2008年1月至2018年12月,将我院48例[男29例、女19例,月龄39.2(3~91)个月]行解剖纠治的ccTGA患者分为2组:双心室解剖纠治组(39例)和1.5心室解剖纠治组(9例),对住院死亡、生存曲线、术后并发症、再干预及心脏功能等进行比较分析。结果双心室解剖纠治组早期死亡3例,早期再干预2例,而1.5心室解剖纠治组无死亡,早期再干预1例,与双心室解剖纠治相比,手术时间、气管插管和ICU时间均显著缩短(P<0.05)。术后随访0.5~10.4年,失访4例,双心室解剖纠治组死亡2例,再干预2例,1年、5年及10年生存率分别为88.2%、84.0%和84.0%。而1.5心室解剖纠治组无死亡,亦无再干预患者。全组其余患者中期随访生活质量均较为满意,心功能分级仅2例为Ⅲ级,其余均为Ⅰ~Ⅱ级。结论依据ccTGA不同的解剖特点采取个体化的解剖纠治策略均可获得满意的手术效果,中期生活质量良好。如果患者合适,1.5心室解剖纠治因其更低的手术死亡率和术后并发症发生率可能将获得更好的治疗效果。

Objective To evaluate the effects of anatomic correction for congenitally corrected transposition of the great arteries(ccTGA)and 10-year follow-up.Methods From January 2008 to December 2018,48 patients with ccTGA who underwent anatomic correction were reviewed.There were 29 males and 19 females with age of 39.2(3-91)months.The cohort was divided into two groups:a biventricular anatomic correction group(39 patients)and a 1.5 ventricular anatomic correction group(9 patients).They were followed for in-hospital mortality,late mortality,long-term survival,freedom from reoperation,and heart function.Results There were 3 early deaths and 2 early re-intervention in the biventricular anatomic correction group,but no death and only one re-intervention in the 1.5 ventricular anatomic correction group.Compared with the biventricular anatomic correction group,the operation time,tracheal intubation and ICU time were significantly reduced or shortened in the 1.5 ventricular anatomic correction group(P<0.05).The patients were followed up for 0.5-10.4 years.Four patients were lost.Two patients died in the biventricular anatomic correction group,and two patients received re-intervention.The 1-year,5-year and 10-year survival rate was 88.2%,84.0%,and 84.0%,respectively.There was no death or intervention in the 1.5 ventricular anatomic correction group.The quality of life of the other patients in the medium-term follow-up was satisfactory.Only 2 patients were classified as gradeⅢin cardiac function,and the other patients were classified as gradeⅠ-Ⅱ.Conclusion According to the different anatomic characteristics of ccTGA,the individualized strategy of anatomic correction can achieve satisfactory surgical results,and the medium-term quality of life was good.Especially,1.5 ventricular anatomic correction may obtain better therapeutic effects because of its lower operative mortality and less postoperative complications.