Nembhard Wendy N, Bourke Jenny, Leonard Helen, Eckersley Luke, Li Jingyun, Bower Carol
Division of Birth Defects Research, Department of Pediatrics, College of Medicine, University of Arkansas for Medical Sciences, Arkansas Children's Hospital Research Institute, Arkansas.
Telethon Kids Institute, University of Western Australia, Western Australia, Australia.
Birth Defects Res A Clin Mol Teratol. 2016 Dec;106(12):1016-1031. doi: 10.1002/bdra.23572. Epub 2016 Nov 1.
Australian Aboriginal children have increased infant and childhood mortality compared with Caucasian children, but their mortality related to congenital heart defects (CHDs) throughout life is unknown.
We conducted a retrospective cohort study using data on 8,110 live born, singleton infants with CHDs born January 1980 to December 2010 from the Western Australian Register of Developmental Anomalies. Vital status was determined from death and medical records. Data for infants with chromosomal anomalies (except Down syndrome) were excluded. Kaplan-Meier Product-Limit estimates and 95% confidence intervals (CIs) were computed by Aboriginality. Hazard ratios (HRs) and 95% CIs were calculated from multivariable Cox-Proportional Hazard Regression models.
Aboriginal children had lower survival than Caucasians for all CHDs combined but most notably during the neonatal period for functional single ventricle (50.0% vs. 86.1%; p = 0.015) and during the postneonatal period for tetralogy of Fallot (87.0% vs. 97.4%; p = 0.021) and atrioventricular septal defect (60.0% vs. 94.6%; p = 0.010). After adjusting for covariates except remoteness and socioeconomic status (SES), Aboriginal children with all CHDs combined (HR = 1.4; 95% CI, 1.0-1.9), with transposition of the great arteries (HR = 4.3; 95% CI, 1.0-18.9) or functional single ventricle (HR = 8.6; 95% CI, 1.3-57.9) had increased risk of mortality compared with Caucasian children. When remoteness and SES were included, the risks were not statistically significant.
Long-term survival was lower for Aboriginal children with CHDs, and Aboriginal children with specific CHD phenotypes had increased risk of mortality throughout life. Increased risk may be due to SES and environmental factors. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:1016-1031, 2016. © 2016 Wiley Periodicals, Inc.
与白种儿童相比，澳大利亚原住民儿童的婴儿期和儿童期死亡率有所上升，但他们一生中与先天性心脏病（CHD）相关的死亡率尚不清楚。
我们进行了一项回顾性队列研究，使用了西澳大利亚发育异常登记处1980年1月至2010年12月出生的8110例患有先天性心脏病的活产单胎婴儿的数据。通过死亡和医疗记录确定生命状态。排除染色体异常（唐氏综合征除外）婴儿的数据。按原住民身份计算Kaplan-Meier乘积限估计值和95%置信区间（CI）。从多变量Cox比例风险回归模型计算风险比（HR）和95%CI。
所有先天性心脏病合并患者中，原住民儿童的生存率低于白种儿童，但最明显的是在新生儿期功能性单心室（50.0%对86.1%；p = 0.015）以及新生儿后期法洛四联症（87.0%对97.4%；p = 0.021）和房室间隔缺损（60.0%对94.6%；p = 0.010）。在对除偏远程度和社会经济地位（SES）之外的协变量进行调整后，所有先天性心脏病合并患者的原住民儿童（HR = 1.4；95%CI，1.0 - 1.9）、大动脉转位（HR = 4.3；95%CI，1.0 - 18.9）或功能性单心室（HR = 8.6；95%CI，1.3 - 57.9）与白种儿童相比，死亡风险增加。当纳入偏远程度和SES时，风险无统计学意义。
患有先天性心脏病的原住民儿童长期生存率较低，患有特定先天性心脏病表型的原住民儿童一生中死亡风险增加。风险增加可能归因于社会经济地位和环境因素。《出生缺陷研究（A部分）》，2016年。©2016威利期刊公司。《出生缺陷研究（A部分）》106:1016 - 1031，2016年。©2016威利期刊公司。
