Department of Paediatrics and Adolescent Medicine, Lillebaelt Hospital University Hospital of Southern Denmark Kolding Denmark.
Department of Regional Health Research University of Southern Denmark Odense Denmark.
J Am Heart Assoc. 2023 Dec 19;12(24):e029871. doi: 10.1161/JAHA.122.029871. Epub 2023 Dec 18.
The purpose of this study was to evaluate the timing of the first cardiac surgery, the number of cardiac surgeries performed, and 30-day postoperative mortality rate for children with severe congenital heart defects (sCHDs) in their first 5 years of life.
This was a population-based data linkage cohort study linking information from 9 European congenital anomaly registries to vital statistics and hospital databases. Data were extracted for 5693 children with sCHDs born from 1995 to 2004. Subgroup analyses were performed for specific types of sCHD. Children with sCHDs underwent their first surgical intervention at a median age of 3.6 (95% CI, 2.6-4.5) weeks. The timing of the first surgery for most subtypes of sCHD was consistent across Europe. In the first 5 years of life, children with hypoplastic left heart underwent the most cardiac surgeries, with a median of 4.4 (95% CI, 3.1-5.6). The 30-day postoperative mortality rate in children aged <1 year ranged from 1.1% (95% CI, 0.5%-2.1%) for tetralogy of Fallot to 23% (95% CI, 12%-37%) for Ebstein anomaly. The 30-day postoperative mortality rate was highest for children undergoing surgery in the first month of life. Overall 5-year survival for sCHD was <90% for all sCHDs, except transposition of the great arteries, tetralogy of Fallot, and coarctation of the aorta.
There were no major differences among the 9 regions in the timing, 30-day postoperative mortality rate, and number of operations performed for sCHD. Despite an overall good prognosis for most congenital heart defects, some lesions were still associated with substantial postoperative death.
本研究旨在评估儿童在生命的前 5 年内首次接受心脏手术的时间、进行的心脏手术数量以及 30 天术后死亡率，这些儿童患有严重先天性心脏缺陷（sCHD）。
这是一项基于人群的数据分析链接队列研究，将来自 9 个欧洲先天性异常登记处的信息与生命统计和医院数据库相链接。研究提取了 1995 年至 2004 年期间出生的 5693 名患有 sCHD 的儿童的数据。对特定类型的 sCHD 进行了亚组分析。患有 sCHD 的儿童中位数年龄为 3.6（95%CI，2.6-4.5）周接受首次手术干预。大多数欧洲 sCHD 亚型的首次手术时间基本一致。在生命的前 5 年中，左心发育不全的儿童接受的心脏手术最多，中位数为 4.4（95%CI，3.1-5.6）。<1 岁儿童的 30 天术后死亡率范围从法洛四联症的 1.1%（95%CI，0.5%-2.1%）到 Ebstein 畸形的 23%（95%CI，12%-37%）。在生命的第一个月接受手术的儿童中，30 天术后死亡率最高。除大动脉转位、法洛四联症和主动脉缩窄外，所有 sCHD 的 5 年总生存率均<90%。
9 个地区在 sCHD 的时间、30 天术后死亡率和手术数量方面没有重大差异。尽管大多数先天性心脏病的总体预后良好，但某些病变仍然与大量术后死亡相关。
