室间隔缺损修补术后继发性主动脉瓣下狭窄的单中心回顾性研究

Secondary subaortic stenosis following ventricular septal defect closure:A retrospective study in a single center

目的总结室间隔缺损患者术后继发性主动脉瓣下狭窄(secondary subaortic stenosis,SSS)的临床经验,探究其潜在发生机制。方法纳入2008—2019年在阜外医院行单纯开胸室间隔缺损修补手术,术前无左室流出道梗阻,但术后出现SSS并于阜外医院进行二次手术的0~18岁患者。回顾性收集患者的临床资料,分析室间隔缺损修补术后出现SSS的形态学特征、再次干预方式及随访结果。结果共纳入6例患者,其中女2例、男4例。首次室间隔缺损修补手术中位年龄9个月(1个月~3岁)。首次手术后2.9年(1~137个月)患者被诊断为SSS,2例患者在确诊后立即行二次手术,其余4例等待1.2年(6~45个月)后行瓣下狭窄手术治疗。最常见的室间隔缺损外科术后SSS为隔膜型,新生隔膜位于左室流出道并呈环形,小部分沿室间隔补片生长。SSS术后中位随访时间8.1(7.3~8.9)年,所有患儿均未复发左室流出道梗阻。结论无论是单纯室间隔缺损外科修补还是合并其他心脏畸形的先天性心脏病外科手术,术后长期随访复查、及时干预,都是阻止主动脉瓣反流或左室流出道梗阻进展的最佳方法。

Objective To summarize the characteristics of children diagnosed with secondary subaortic stenosis after the surgical closure for ventricular septal defect and explore its potential mechanism.Methods We retrospectively collected patients aged from 0 to 18 years,who underwent ventricular septal defect closure and developed secondary subaortic stenosis,and subsequently received surgical repair from 2008 to 2019 in Fuwai Hospital.Their surgical details,morphological features of the subaortic stenosis,and the follow-up information were analyzed.Results Six patients,including 2 females and 4 males,underwent the primary ventricular septal defect closure at the median age of 9 months(ranging from 1 month to 3 years).After the first surgery,patients were diagnosed with secondary subaortic stenosis after 2.9 years(ranging from 1 to 137 months).Among them,2 patients underwent the second surgery immediately after diagnosis,and the other 4 patients waited 1.2 years(ranging from 6 to 45 months)for the second surgery.The most common type of the secondary subaortic stenosis after ventricular septal defect closure was discrete membrane,which located underneath the aortic valve and circles as a ring.In some patients,subaortic membrane grew along with the ventricular septal defect closure patch.During the median follow-up of 8.1 years(ranging from 7.3 to 8.9 years)after the sencond surgery,all patients recovered well without any recurrence of left ventricular outflow tract obstruction.Conclusion Regular and persistent follow-up after ventricular septal defect closure combining with or without other cardiac malformation is the best way to diagnose left ventricular outflow tract obstruction in an early stage and stop the progression of aortic valve regurgitation.