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发展中国家儿童重症先天性心脏病的外科治疗。

Surgery for severe congenital heart diseases in children from developing nations.

作者信息

Lacour-Gayet Francois, Gouton Marielle, Bical Olivier, Lucet Vincent, Roussin Regine, Leca Francine

机构信息

Mécénat Chirurgie Cardiaque Foundation, Paris, France.

Department of Pediatric Cardiology, Hopital Montsouris, Paris, France.

出版信息

J Thorac Cardiovasc Surg. 2022 Feb;163(2):413-423. doi: 10.1016/j.jtcvs.2021.04.088. Epub 2021 May 14.

DOI:10.1016/j.jtcvs.2021.04.088
PMID:34053740
Abstract

BACKGROUND

Children with severe congenital heart disease (CHD) are rarely treated in developing countries and have very little to no chance to survive in their local environment. Mécénat Chirurgie Cardiaque (MCC) flies to France children with CHD from developing countries. This report focuses on the early, mid, and late outcomes of 531 children with severe CHD sent to MCC for surgery from 1996 to 2019.

METHODS

The inclusion criteria were based on diagnosis and not on procedure. MCC is present in 66 countries and has developed a robust staff, including 12 permanent employees and 700 volunteers, with 350 host families based in France, 120 local correspondents, and 100 local physicians. Since 1996, MCC has organized a basic training of local pediatric cardiologists yearly, offering a free 1-month training course. Over time, MCC could count on a pool of doctors trained in basic pediatric cardiology. Flights were secured by the Aviation Sans Frontieres Foundation. Nine French centers performed the surgeries. A robust follow-up was conducted in all the nations where MCC operates.

RESULTS

The most frequent pathologies were single ventricle (n = 126), double-outlet right ventricle (n = 116), pulmonary atresia with ventricular septal defect (n = 68), transposition of the great arteries with ventricular septal defect and transposition of the great arteries with intact ventricular septum (n = 61), arterial trunk (n = 39), transposition of the great arteries with ventricular septal defect and left ventricle outflow tract obstruction (n = 35), complete atrioventricular septal defect (n = 18), congenitally corrected transposition of the great arteries (n = 16), and so on. The median age was 5.4 years (range, 1 month-26 years). The mean perioperative mortality was 5.5% (29 out of 531) (95% confidence limit, 3.5%-7.4%). The follow-up was 91.3%, with a mean follow-up of 5.1 years. The global actuarial survival at 5, 10, and 15 years was, respectively, 85%, 83%, and 74%. There was a significant higher late mortality for patients surviving only with a Blalock-Taussig shunt (P = .001).

CONCLUSIONS

Operating on 531 children with severe CHD from developing nations was achieved with satisfactory early and long-term results. Children with severe CHD are rarely operated on in developing nations. Programs like MCC's offer a viable option to save these children born with severe CHD.

摘要

背景

患有严重先天性心脏病(CHD)的儿童在发展中国家很少能得到治疗,在当地环境下存活的机会微乎其微。心脏外科慈善组织(Mécénat Chirurgie Cardiaque,MCC)将来自发展中国家患有CHD的儿童接到法国接受治疗。本报告聚焦于1996年至2019年被送到MCC接受手术的531例患有严重CHD儿童的早期、中期和晚期治疗结果。

方法

纳入标准基于诊断而非手术方式。MCC在66个国家开展工作,拥有一支强大的团队,包括12名长期雇员和700名志愿者,在法国有350个寄宿家庭、120名当地通讯员和100名当地医生。自1996年以来,MCC每年都会为当地儿科心脏病专家组织基础培训,提供为期1个月的免费培训课程。随着时间的推移,MCC拥有了一批接受过基础儿科心脏病学培训的医生。航班由无国界航空基金会提供保障。9家法国中心进行手术。MCC开展工作的所有国家都进行了有力的随访。

结果

最常见的病变类型为单心室(n = 126)、右心室双出口(n = 116)、室间隔缺损合并肺动脉闭锁(n = 68)、室间隔缺损合并大动脉转位及室间隔完整的大动脉转位(n = 61)、动脉干(n = 39)、室间隔缺损合并大动脉转位及左心室流出道梗阻(n = 35)、完全性房室间隔缺损(n = 18)、先天性矫正型大动脉转位(n = 16)等。中位年龄为5.4岁(范围1个月至26岁)。围手术期平均死亡率为5.5%(531例中有29例)(95%置信区间,3.5% - 7.4%)。随访率为91.3%,平均随访时间为5.1年。5年、10年和15年的总体精算生存率分别为85%、83%和74%。仅通过Blalock-Taussig分流术存活的患者晚期死亡率显著更高(P = 0.001)。

结论

对来自发展中国家的531例患有严重CHD的儿童进行手术,取得了令人满意的早期和长期结果。患有严重CHD的儿童在发展中国家很少接受手术。像MCC这样的项目为拯救这些患有严重CHD的儿童提供了可行的选择。

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