小儿先天性主动脉瓣疾病不同治疗策略的疗效分析：单中心85例的回顾性队列研究

Efficacy of different treatment strategies for congenital aortic valve disease in 85 children from a single center: A retrospective cohort study

目的分析先天性主动脉瓣疾病患儿的各类手术方法的早中期治疗结果,为主动脉瓣狭窄或反流患儿的手术方式提供参考。方法回顾性分析2005年1月至2018年12月在复旦大学附属儿科医院进行手术治疗的85例主动脉瓣疾病患儿的临床资料,男64例、女21例,平均年龄45个月(5 d^15岁)。其中18例行主动脉瓣球囊扩张成形术(BAV),8例行主动脉瓣外科交界切开成形术(SAV),27例行主动脉瓣自体心包修补术,16例行机械瓣主动脉瓣置换术,16例行Ross手术。随访(6.25±2.76)年,分析不同手术术后再干预情况和患儿生存状况。结果85例患儿死亡3例(3.5%),再手术17例(20.0%)。采用SAV、BAV、主动脉瓣自体心包修补术、机械瓣主动脉瓣置换术、Ross手术患儿5年生存率分别为87.4%、88.9%、100.0%、100.0%、100.0%,差异无统计学意义(P>0.05);5年免再手术率分别为44.4%、18.4%、100.0%、66.9%、80.5%,差异具有统计学意义(P<0.05)。结论先天性主动脉瓣狭窄或反流手术需要根据患儿瓣叶组织病变情况采取手术方式。对于严重病变患儿,首次干预推荐SAV。先天性主动脉瓣狭窄手术中SAV、BAV均为姑息性手术,中远期需进一步评估以及再干预可能;Ross手术和机械瓣主动脉瓣置换术有较低的再干预率,中远期随访表明疗效确切;主动脉瓣自体心包修补术有望成为延期或替代Ross手术和瓣膜置换术的方法。

Objective To analyze the early-and middle-term prognosis of various surgical methods in children with congenital aortic valve diseases,to provide reference for surgical methods in children with aortic valve stenosis or regurgitation.Methods The clinical data of 85 children with various aortic valve diseases treated in the Children’s Hospital of Fudan University from January 2005 to December 2018 were retrospectively analyzed.There were 64 males and 21 females,with an average age of 45 months ranging from 5 days to 15 years.Among them 18 patients underwent balloon aortic valvuloplasty(BAV),8 surgical aortic valvotomy(SAV),27 aortic valve autogenous pericardium repair,16 mechanical arterial valve replacement and 16 Ross operation.They were followed up for 6.25±2.76 years.The reintervention and survival status after different operations were analyzed.Results There were 3 deaths and 17 reoperations in 85 children.The 5-year survival rate of the patients with SAV,BAV,aortic valve autogenous pericardium repair,mechanical arterial valve replacement and Ross operation was 87.4%,88.9%,100.0%,100.0%and 100.0%,respectively;there was no statistical difference in the early and middle-term survival rates among various operations(P>0.05).The 5-year free from re-intervention rate of the patients with SAV,BAV,aortic valve autogenous pericardium repair,mechanical arterial valve replacement and Ross operation was 44.4%,18.4%,100.0%,66.9%and 80.5%,respectively;there was a statistical difference in the early and middle-term re-intervention rate among various operations(P<0.05). Conclusion The operation of congenital aortic stenosis or regurgitation needs to be performed according tothe pathological changes of the valvular tissues. For children with severe lesions, SAV is recommended for the firstintervention. For congenital aortic stenosis, SAV and BAV are both palliative operations which need further evaluationand re-intervention. Ross operation and mechanical arterial valve replacement have low re-intervention rate, and themiddle- and long-term follow-up shows that the effect is accurate. Aortic valve autogenous pericardium repair is expectedto become a method to delay or replace Ross operation and valve replacement.