全腔静脉-肺动脉连接术治疗功能性单心室合并内脏异位综合征的倾向性评分匹配研究

Outcomes of total cavopulmonary connection in the treatment of functional single ventricle with heterotaxy syndrome:A propensity score matching study

目的总结全腔静脉-肺动脉连接术(total cavopulmonary connection,TCPC)治疗功能性单心室合并内脏异位综合征(heterotaxy syndrome,HS)的临床经验和结果。方法回顾性分析2004—2021年于广东省人民医院行TCPC合并HS的功能性单心室患者(HS组)的临床资料,分析患者术后并发症、远期生存率以及影响生存的相关因素。将术后早期和晚期结果与匹配的同时期非HS患者(非HS组)进行比较。结果倾向性评分匹配前HS组纳入患者55例,其中男42例、女13例,中位年龄6.0(4.2,11.8)岁,中位体重17.0(14.2,28.8)kg。右房异构53例,左房异构2例。一期完成TCPC者8例。TCPC手术类型包括心外管道39例、心内-心外管道14例、主肺动脉-下腔静脉直接吻合2例。术后27例发生感染,19例肝功能损害,11例急性肾损伤。早期死亡5例,中位随访时间94.7(64.3,129.8)个月,1年、5年、10年总生存率为87.2%、85.3%、74.3%。倾向性评分匹配后HS组45例,非HS组81例。与非HS组相比,HS组手术时间、机械通气时间延长,术后感染率显著增加(P<0.05),术后总生存率明显下降,10年生存率较非HS组低12.9%。多因素Cox回归分析提示,无脾为死亡的危险因素[HR=8.98,95%CI(1.86,43.34),P=0.006]。结论与非HS患者相比,合并HS患者TCPC术后的生存情况较差,无脾是影响该类患者生存的独立危险因素。

Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection(TCPC)in the treatment of functional single ventricle combined with heterotaxy syndrome(HS).Methods A retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC(a HS group)in Guangdong Provincial People's Hospital between 2004 and 2021.The analysis focused on postoperative complications,long-term survival rates,and identifying factors associated with patient survival.Early and late postoperative outcomes were compared with matched non-HS patients(a non-HS group).Results Before propensity score matching,55 patients were collected in the HS group,including 42 males and 13 females,with a median age of 6.0(4.2,11.8)years and a median weight of 17.0(14.2,28.8)kg.Among the patients,there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism.Eight patients underwent TCPC in one stage.TCPC procedures included extracardiac conduit(n=39),intracardiac-extracardiac conduit(n=14),and direct cavopulmonary connection(n=2).Postoperative complications included infections in 27 patients,liver function damage in 19 patients,and acute kidney injury in 11 patients.There were 5 early deaths.The median follow-up time was 94.7(64.3,129.8)months.The 1-year,5-year,and 10-year survival rates were 87.2%,85.3%,and 74.3%,respectively.After propensity score matching,there were 45 patients in the HS group and 81 patients in the non-HS group.Compared to the non-HS group,those with HS had longer surgical and mechanical ventilation time,higher infection rates(P<0.05),and a 12.9%lower 10-year survival rate.Multivariate Cox regression analysis identified asplenia was a risk factor for mortality(HR=8.98,95%CI 1.86-43.34,P=0.006).Conclusion Compared to non-HS patients,patients with HS have lower survival rates after TCPC,and asplenia is an independent risk factor for the survival of these patients.