继发于先天性心脏病术后的主动脉瓣下狭窄手术矫治分析

目的 总结少见病例——继发于先天性心脏病（congenital heart disease，CHD）手术后主动脉瓣下狭窄（secondary subaortic stenosis，SSS）的手术疗效，并分析导致SSS发生的原因。 方法 回顾性分析2008年6月1日至2012年12月31日期间广东省人民医院对SSS进行手术矫治19例患者的临床资料，其中男10例，女9例。CHD类型主要包括右心室双出口、室间隔缺损、部分性房室间隔缺损等。患者矫治CHD时的年龄：4个月（15 d～5岁11个月）；行主动脉瓣下狭窄矫治时的年龄：5岁3个月（1岁～15岁3个月）。患者行SSS矫治与CHD矫治的相隔时间：4年10个月（8个月～13年11个月）。 结果 所有患者均行主动脉瓣下狭窄矫治术，无术中死亡；体外循环时间79 （39～172） min，主动脉阻断时间42 （22～124） min。术后住院时间7 （5～9） d。1例术后需安装永久性心脏起搏器。所有患者均随访。随访时间1年10个月（5个月～4年4个月）。所有SSS患者术后无临床症状，心功能正常，随访过程中无死亡。1例患者随访过程中左心室流出道狭窄逐渐加重，再次行主动脉瓣下狭窄矫治术。 结论继发先天性心脏病术后出现主动脉瓣下狭窄极少见，其发生原因考虑CHD矫治后左心室流出道血流动力学紊乱造成，其发生时间与继发先心病手术类型无法预测，主动脉瓣下狭窄矫治术对SSS矫治简单、有效，但术后左心室流出道仍有可能再次狭窄并加重。

Surgical Results of Secondary Subaortic Stenosis after Congenital Heart Disease Operations

Objective To summarize surgical results of secondary subaortic stenosis （SSS） after congenital heart disease （CHD） operations,and analyze the pathogenesis of SSS. Methods We retrospectively analyzed clinical data of 19 patients who underwent surgical repair for SSS in Guangdong General Hospital from 1st June 2008 to 31st December 2012. There were 10 males and 9 females. CHD types included double outlet right ventricle,ventricular septal defect and partial atrioventricular septal defect,et al. The median age of the patients when they received their first CHD operations was 4 months （15 days to 5 year and 11 months）. The median age of the patients when they received SSS repair was 5 years and 3 months （1 year to 15 years and 3 months）. The median time interval from CHD operation to SSS repair was 4 years and 10 months （8 months to 13 years and 11 months）. Results All the patients successfully received their SSS repair. There was no surgical death in this study. Median cardiopulmonary bypass time was 79 （39 to 172） minutes,and median aortic cross-clamp time was 42 （22 to 124） minutes. Median postoperative hospital stay was 7 （5 to 9） days. Postoperatively, 1 patient required permanent pacemaker implantation. All the patients were followed up after discharge for a median durationof 1 year and 10 months （5 months to 4 years and 4 months） . During follow-up, none of the patients had any clinical symptom,their heart function was normal,and there was no late death. One patient received another subaortic stenosis repair for gradually aggravating left ventricular outflow tract stenosis. Conclusions SSS is very rare after CHD operations. The pathogenesis of SSS is perhaps related to abnormal blood flows in the left ventricular outflow tract after CHD operations. The pathogenesis time of SSS and types of CHD leading to SSS cannot be predicted. Subaortic stenosis repair is a simple and safe procedure for SSS,but postoperatively left ventricular outflow tract stenosis may appear and aggravate again.