1980年至2010年西澳大利亚土著和高加索儿童先天性心脏缺陷的25年生存率

Q Medicine Birth defects research. Part A, Clinical and molecular teratology Pub Date : 2016-11-01 DOI:10.1002/bdra.23572

Wendy N. Nembhard, Jenny Bourke, Helen Leonard, Luke Eckersley, Jingyun Li, Carol Bower

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引用次数: 4

摘要

澳大利亚土著儿童的婴儿和儿童死亡率比白种人儿童高，但其一生中与先天性心脏缺陷(CHDs)相关的死亡率尚不清楚。方法:我们对1980年1月至2010年12月出生的8110名CHDs活产单胎婴儿进行了回顾性队列研究，数据来自西澳大利亚发育异常登记处。根据死亡和医疗记录确定生命状况。排除了染色体异常婴儿(唐氏综合征除外)的数据。Kaplan-Meier产品极限估计和95%置信区间(ci)由Aboriginality计算。通过多变量cox -比例风险回归模型计算风险比(hr)和95% ci。结果原住民儿童合并冠心病的生存率均低于白种人，但在新生儿期的单心室功能表现最为明显(50.0% vs. 86.1%;p = 0.015)和新生儿后期法洛四联症(87.0% vs. 97.4%;P = 0.021)和房室间隔缺损(60.0% vs. 94.6%;P = 0.010)。在调整除偏远地区和社会经济地位(SES)以外的协变量后，所有冠心病的原住民儿童合并(HR = 1.4;95% CI, 1.0-1.9)，伴有大动脉转位(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)与白种人儿童相比死亡风险增加。当包括偏远和SES时，风险无统计学意义。结论土著儿童冠心病患者的长期生存率较低，具有特定冠心病表型的土著儿童终生死亡风险较高。增加的风险可能是由于SES和环境因素。出生缺陷研究(上)，2016。©2016 Wiley期刊公司出生缺陷研究(A辑)106:1016-1031,2016。©2016 Wiley期刊公司

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Twenty-five–year survival for aboriginal and caucasian children with congenital heart defects in Western Australia, 1980 to 2010

Background

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.

Methods

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.

Results

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.

Conclusion

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.

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