完全性大动脉转位合并左心室流出道狭窄患儿接受大动脉调转术后的预后情况和相关风险因素分析

The prognosis of the complete transposition of great arterial with left ventricular outflow tract obstruction after arterial switch operation

目的研究接受大动脉调转术(ASO)的完全性大动脉转位合并左心室流出道狭窄(TGA/LVOTO)患儿的病例特征,分析患儿术后不良事件的相关危险因素。方法回顾性分析39例TGA/LVOTO患儿接受ASO手术后的不良预后事件,包括术后死亡、再次手术、主动脉瓣关闭不全(AVI)、二尖瓣关闭不全(MVI)、左心室流出道再狭窄(re-LVOTO)和室间隔缺损残余分流,及其相关的风险因素。结果 39例TGA/LVOTO患儿,男31例,女8例。肺动脉瓣环(PV)内径平均Z值0.6,左心室流出道峰值压差均值31.6 mmHg(1 mmHg=0.133 kPa)。平均随访15.9个月,早期死亡1例,术中体外循环时间较长(P=0.034)和术后早期死亡独立相关;早期再干预患儿4例;术后AVI患儿15例,PV内径Z值较大(P=0.026)和术后AVI独立相关;术后MVI患儿7例,瓣下水平的LVOTO(P=0.001)和术后MVI独立相关。术后室间隔残余分流4例,多变量分析显示肌部室间隔缺损(P=0.021)患儿更易出现术后残余分流(以膜周部缺损为基准);6例发生了re-LVOTO,手术时年龄较大(P=0.029)、多部位LVOTO(P=0.024)为术后re-LVOTO的独立危险因素。结论若TGA患儿的LVOTO可以被手术疏通,则接受ASO术后早期病死率低,早期再干预主要原因为手术并发症,术后re-LVOTO率逐年升高,但远期再手术率很低,总体预后良好。

To analyze the clinical characteristics of complete transposition of great arterial with left ventricular outflow tract obstruction(TGA/LVOTO) patients who received arterial switch operation(ASO), and further evaluated the risk factors of postoperative adverse events. Methods Retrospectively evaluated the adverse events(including postoperative mortality, reoperation, aortic valve insufficiency, re-LVOTO and mitral valve insufficiency) and the related risk factors of 39 TGA/LVOTO patients after ASO. Results 39 TGA/LVOTO patients were included, the mean Z value of PV was 0.6, the mean peak LVOT gradient was 31.6 mmHg(1 mmHg=0.133 kPa). The mean follow up time was 15.9 months, during the follow up, 1 patient had early mortality, and CPB time(P=0.034) was associated with early mortality;4 patients had early reintervention;15 patients had AVI, and larger PV Z value(P=0.026) was associated with postoperative AVI;7 patients had MVI, and subvalvar level LVOTO(P=0.001) was associated postoperative MVI;6 patients had re-LVOTO, and older age at operation(P=0.029), multi-level LVOTO(P=0.024) were associated with postoperative re-LVOTO. Conclusion If the obstruction of LVOT can be repaired by surgery, TGA/LVOTO patients had a satisfied prognosis after ASO with relief of LVOTO, the postoperative early mortality was very low, and although the probability of re-LVOTO was increased with time, the long-term reintervention probability was very low.