双心室矫治室间隔缺损远离型右室双出口单中心15年经验

目的总结单中心双心室矫治室间隔缺损远离型右室双出口的15年经验,探讨双心室矫治的合适方法,分析再手术的危险因素。方法回顾性分析2005~2019年于我院连续入组162例接受双心室矫治的室间隔缺损远离型右室双出口患儿的临床资料。根据内隧道建立的路径将患儿分为两组:110例患儿术中行室间隔缺损连接至主动脉内隧道A组,男75例、女35例,平均年龄(3.6±3.2)岁];52例患儿行室间隔缺损连接至肺动脉内隧道B组,男30例、女22例,平均年龄(2.8±2.7)岁]。为了建立通畅的心室内隧道,同期进行的操作包括室间隔缺损扩大、圆锥肌肉切除、三尖瓣腱索或乳头肌转移等。结果全组行双心室矫治的患儿早期死亡9例(5.6%),早期心室内隧道梗阻6例(3.7%)。经过(7.5±7.0)年的随访,8例(4.9%)患儿发生晚期死亡,A组的1年、5年、10年、15年生存率分别为92.7%、91.1%、91.1%、85.4%,B组分别为92.2%、85.2%、85.2%、85.2%,两组差异无统计学意义(P=0.560)。随访发现10例(6.2%)迟发性心室内隧道梗阻,8例接受了再次手术。两组比较A组患儿有更多的迟发性心室内隧道梗阻(A组9例vs.B组1例,P=0.017)及总体心室内隧道梗阻(A组15例vs.B组1例,P=0.001)。两组患儿的早期死亡率和晚期死亡率差异无统计学意义(P=0.386、0.223)。A组中同期进行三尖瓣操作46例,其中1例发生左室流出道梗阻,是否同期行三尖瓣操作的术后左室流出道梗阻发生率差异具有统计学意义(1/46 vs.15/64,P=0.004),并不会造成三尖瓣反流或狭窄。Rastelli术后患儿因右室流出道病变再手术率明显高于REV手术和双根部调转术,两者差异具有统计学意义(5/14 vs.0/38,P<0.001)。结论双心室矫治室间隔缺损远离型右室双出口可以取得令人满意的远期结果。室间隔缺损连接至主动脉内隧道较室间隔缺损连接至肺动脉内隧道左室流出道梗阻发生率更高。同期进行三尖瓣处理可降低内隧道梗阻发生率。

Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect:15-year experience of a single center

Objective To explore the appropriate method of biventricular repair and analyze the risk factors for reoperation,by summarizing the 15-year treatment experience of biventricular repair for double outlet right ventricle with non-committed ventricular septal defect(DORVncVSD).Methods Clinical data of 162 consecutive patients with DORVncVSD who had biventricular repair from 2005 to 2019 in our center were retrospectively analyzed.The children were divided into two groups according to the path of intracardiac tunnel:110 patients with ventricular septal defect rerouted to the aorta were recruited into a group A(75 males and 35 females aged 3.6±3.2 years);52 patients with ventricular septal defect rerouted to the pulmonary artery were into a group B(30 males and 22 females aged 2.8±2.7 years).In order to establish a smooth intracardiac tunnel,enlargement of VSD,the resection of conus muscle and the transfer of tricuspid tendon or papillary muscle,etc were performed at the same time.Results In the patients with biventricular repair,there were 9(5.6%)early deaths and 6(3.7%)early intracardiac baffle obstructions.During the follow-up of 7.5±7.0 years,and 8(4.9%)late deaths occurred.The 1-year,5-year,10-year and 15-year survival rates of the group A were 92.7%,91.1%,91.1%,85.4%,respectively and those of the group B were 92.2%,85.2%,85.2%,85.2%,respectively.The difference between the two groups was not statistically significant(P=0.560).The follow-up results showed that 10(6.2%)patients had late-onset intracardiac tunnel obstruction,and 8 patients underwent reoperation.There were more late-onset intracardiac tunnel obstruction patients and overall intracardiac tunnel obstruction patients in the group A than those in the group B(9 patients vs.1 patient,P=0.017;15 patients vs.1 patient,P=0.001).No significant difference of early mortality and late mortality was noted for the group A(P=0.386)and the group B(P=0.223).Also it was noted that performing tricuspid valve operation at the same time in the group A had a significant impact to reduce the occurrence rate of intracardiac obstruction(1/46 vs.15/64,P=0.004),without any tricuspid regurgitation or stenosis.The reoperation rate of patients with Rastelli after right ventricular outflow tract lesions was significantly higher than that of REV surgery and double root replacement surgery(5/14 vs.0/38,P<0.001).Conclusion The effect of biventricular repair for DORVncVSD is satisfactory.And concomitant tricuspid procedures can help reduce the occurrence of intracardiac obstructions.Reconstruction of right ventricular outflow tract with biological valved conduit is a risk factor for reoperation.