title: Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome.
abstract: BACKGROUND
  Most studies have not demonstrated improved survival after prenatal diagnosis of critical congenital heart disease, including hypoplastic left heart syndrome (HLHS). However, the effect of delivery near a cardiac surgical center (CSC), the recommended action after prenatal diagnosis, on HLHS mortality has been poorly investigated.
  
  
  RESULTS
  Using Texas Birth Defects Registry data, 1999 through 2007, which monitored >3.4 million births, we investigated the association between distance (calculated driving time) from birth center to CSC and neonatal mortality in 463 infants with HLHS. Infants with extracardiac birth defects or genetic disorders were excluded. The associations between prenatal diagnosis, CSC HLHS volume, and mortality were also examined. Neonatal mortality in infants born <10 minutes from a CSC was 21.0%, 10 to 90 minutes 25.2%, and >90 minutes 39.6% (P for trend <0.001). Prenatal diagnosis alone was not associated with improved survival (P=0.14). In multivariable analysis, birth >90 minutes from a CSC remained associated with increased mortality (odds ratio, 2.03; 95% confidence interval, 1.19-3.45), compared with <10 minutes. In subanalysis, birth >90 minutes from a CSC was associated with higher pretransport mortality (odds ratio, 6.69; 95% confidence interval, 2.52-17.74) and birth 10 to 90 minutes with higher presurgical mortality (odds ratio, 4.45; 95% confidence interval, 1.17-17.00). Higher surgical mortality was associated with lower CSC HLHS volume (odds ratio per 10 patients, 0.88; 95% confidence interval, 0.84-0.91).
  
  
  CONCLUSIONS
  Infants with HLHS born far from a CSC have increased neonatal mortality, and most of this mortality is presurgical. Efforts to improve prenatal diagnosis of HLHS and subsequent delivery near a large volume CSC may significantly improve neonatal HLHS survival.
authors.affiliation: Department of Pediatrics and Cardiovascular Research Institute, Baylor College of Medicine, Houston, TX (S.A.M., D.J.P.); Texas Department of State Health Services, Austin, TX (M.K.E., M.A.C.); Dan L. Duncan Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, TX (C.G.M.); Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX (D.E.F.); and the Department of Epidemiology & Biostatistics, University of South Florida, Tampa, FL (W.N.N.).
authors.forename: Shaine A
authors.initials: SA
authors.lastname: Morris
authors.forename: Mary K
authors.initials: MK
authors.lastname: Ethen
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Penny
authors.forename: Mark A
authors.initials: MA
authors.lastname: Canfield
authors.forename: Charles G
authors.initials: CG
authors.lastname: Minard
authors.forename: David E
authors.initials: DE
authors.lastname: Fixler
authors.forename: Wendy N
authors.initials: WN
authors.lastname: Nembhard
mesh_terms: D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D007226:Infant Mortality; D007231:Infant, Newborn; D008297:Male; D017063:Outcome Assessment, Health Care; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000706:statistics & numerical data*; D012042:Registries / Q000706:statistics & numerical data; D012189:Retrospective Studies; D012307:Risk Factors; D012959:Socioeconomic Factors; D013781:Texas / Q000453:epidemiology; D013903:Thoracic Surgery / Q000458:organization & administration / Q000706:statistics & numerical data*; D061665:Time-to-Treatment / Q000706:statistics & numerical data
keywords: heart defects, congenital; hypoplastic left heart syndrome; mortality; population; prenatal diagnosis