Long-Term Survival and Causes of Death in Children with Trisomy 21 After Congenital Heart Surgery.
Long-Term Survival and Causes of Death in Children with Trisomy 21 After Congenital Heart Surgery.
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DOI：
10.1016/j.jpeds.2020.12.058
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发表时间：
2021-04
期刊：
影响因子：
--
通讯作者：
Setty SP
中科院分区：
文献类型：
--
作者：
Peterson JK;Kochilas LK;Knight J;McCracken C;Thomas AS;Moller JH;Setty SP
评估21三体(T21)人群先天性心脏病(CHD)术后的长期无移植生存和死亡原因，并与整倍体患者进行比较。这是一项来自儿科心脏护理联盟的回溯性队列研究，丰富了从国家死亡指数和器官采购和移植网络为有足够直接识别符的患者前瞻性收集的数据。生成Kaplan-Meier生存图，并使用多变量Cox比例风险模型检验T21和1：1匹配的具有相似冠心病的整倍体患者的死亡率的危险因素。对3376名符合纳入标准的T21患者(75,155人年)进行了长期生存分析。T21患者的30年生存率从室间隔缺损者的92.1%到复杂房室管的65.3%不等。在这些患者中，2185名T21患者与一名整倍体患者成功配对。经过22.86年(IQR 19.45-27.14年)的中位随访，T21组有213例死亡(9.7%)，而整倍体对照组有123例(5.6%)死亡。在调整了年龄、性别、年龄、先天性心脏病复杂性和最初的缓解措施后，T21患者的冠心病相关死亡风险比高出1.34倍(95%CI为0.92~1.97，P=.127)。心脏外科治疗后T21患者的CHD相关死亡率与整倍体对照指标相当。患有T21的儿童需要终身监测与他们的染色体异常相关的共生疾病。
To evaluate long-term transplant-free survival and causes of death in the Trisomy 21 (T21) population after surgery for congenital heart disease (CHD) in comparison with euploidic patients. This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, enriched with prospectively collected data from the National Death Index and the Organ Procurement and Transplantation Network for patients with sufficient direct identifiers. Kaplan-Meier survival plots were generated and multivariable Cox proportional hazards models were used to examine risk factors for mortality between T21 and 1:1 matched euploidic patients with a comparable CHD. Long-term survival analysis was completed for 3,376 patients with T21 (75,155 person-years) who met inclusion criteria. Thirty-year survival for patients with T21 ranged from 92.1% for ventricular septal defect to 65.3% for complex common atrioventricular canal. Of these, 2,185 patients with T21 were successfully matched to a euploidic patient. After a median follow-up of 22.86 years (IQR 19.45–27.14 years), 213 deaths occurred in the T21 group (9.7%), compared with 123 (5.6%) in the euploidic comparators. After adjustment for age, sex, era, congenital heart disease complexity and initial palliation, the hazard ratio of CHD-related mortality was 1.34 times higher in patients with T21 (95% CI 0.92–1.97, P = .127). CHD-related mortality for patients with T21 following cardiac surgical intervention is comparable with euploidic comparators. Children with T21 require lifelong surveillance for co-occurring conditions associated with their chromosomal abnormality.
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影响因子：
2.8
作者：
Duffels, Marielle G. J.;Vis, Jeroen C.;Mulder, Barbara J. M.
通讯作者：
Mulder, Barbara J. M.
影响因子：
2.1
作者：
Beghetti, Maurice;Rudzinski, Andrzej;Zhang, Min
通讯作者：
Zhang, Min
DOI：
10.1046/j.1365-2788.1999.00198.x
发表时间：
1999-06-01
影响因子：
3.6
作者：
Frid, C;Drott, P;Annerén, G
通讯作者：
Annerén, G
影响因子：
158.5
作者：
Galiè, N;Ghofrani, HA;Simonneau, G
通讯作者：
Simonneau, G
影响因子：
--
作者：
Hoashi, Takaya;Miyata, Hiroaki;Takamoto, Shinichi
通讯作者：
Takamoto, Shinichi