title: Twenty-five-year survival for aboriginal and caucasian children with congenital heart defects in Western Australia, 1980 to 2010.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors: 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.
authors: Wendy N
authors: WN
authors: Nembhard
authors: Telethon Kids Institute, University of Western Australia, Western Australia, Australia.
authors: Jenny
authors: J
authors: Bourke
authors: Telethon Kids Institute, University of Western Australia, Western Australia, Australia.
authors: Helen
authors: H
authors: Leonard
authors: Children's Cardiac Centre, Princess Margaret Hospital, Western Australia, Australia.
authors: Luke
authors: L
authors: Eckersley
authors: Division of Birth Defects Research, Department of Pediatrics, College of Medicine, University of Arkansas for Medical Sciences, Arkansas Children's Hospital Research Institute, Arkansas.
authors: Jingyun
authors: J
authors: Li
authors: Telethon Kids Institute, University of Western Australia, Western Australia, Australia.|Western Australian Register of Developmental Anomalies, King Edward Memorial Hospital, Western Australia, Australia.
authors: Carol
authors: C
authors: Bower
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006343:Heart Septal Defects / Q000453:epidemiology* / Q000208:ethnology / Q000401:mortality / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D013771:Tetralogy of Fallot / Q000453:epidemiology* / Q000208:ethnology / Q000401:mortality / Q000473:pathology; D014188:Transposition of Great Vessels / Q000453:epidemiology* / Q000208:ethnology / Q000401:mortality / Q000473:pathology; D014904:Western Australia / Q000453:epidemiology; D044465:White People
keywords: Aboriginal; children; congenital heart defects; long-term; mortality