title: Survival of Children With Hypoplastic Left Heart Syndrome.
abstract: OBJECTIVE
  To examine the survival of infants with hypoplastic left heart syndrome (HLHS) and potential influence of demographic and clinical characteristics on survival using population-based data.
  
  
  METHODS
  Infants with nonsyndromic HLHS (n = 212) born between 1979 and 2005 were identified through the Metropolitan Atlanta Congenital Defects Program. Vital status was ascertained through 2009 based on linkage with vital records. We estimated Kaplan-Meier survival probabilities stratified by select demographic and clinical characteristics.
  
  
  RESULTS
  The overall survival probability to 2009 was 24% and significantly improved over time: from 0% in 1979-1984 to 42% in 1999-2005. Survival probability was 66% during the first week, 27% during the first year of life, and 24% during the first 10 years. Survival of very low and low birth weight or preterm infants and those born in high-poverty neighborhoods was significantly poorer. For children with information on surgical intervention (n = 88), the overall survival was 52%, and preterm infants had significantly poorer survival (31%) compared with term infants (56%). For children who survived to 1 year of age, long-term survival was ∼90%.
  
  
  CONCLUSIONS
  Survival to adolescence of children with nonsyndromic HLHS born in metropolitan Atlanta has significantly improved in recent years, with those born full term, with normal birth weight, or in a low-poverty neighborhood having a higher survival probability. Survival beyond infancy to adolescence is high. A better understanding of the growing population of survivors with HLHS is needed to inform resource planning.
authors.affiliation: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia; College of Allied Health Sciences, Georgia Regents University, Augusta, Georgia;
authors.forename: Csaba
authors.initials: C
authors.lastname: Siffel
authors.affiliation: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia; tcolarusso@cdc.gov.
authors.forename: Tiffany
authors.initials: T
authors.lastname: Riehle-Colarusso
authors.affiliation: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia; Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia; and.
authors.forename: Matthew E
authors.initials: ME
authors.lastname: Oster
authors.affiliation: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia; Department of Medicine, University of Mississippi Medical Center, Jackson, Mississippi.
authors.forename: Adolfo
authors.initials: A
authors.lastname: Correa
mesh_terms: D002648:Child; D005260:Female; D005500:Follow-Up Studies; D005845:Georgia; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality* / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D008297:Male; D011336:Probability; D011379:Prognosis; D012307:Risk Factors; D016019:Survival Analysis