title: Outcomes after listing for primary transplantation for infants with unoperated-on non-hypoplastic left heart syndrome congenital heart disease: a multi-institutional study.
abstract: BACKGROUND
  Although heart transplantation has been used as the primary therapy for congenital heart lesions in infants other than hypoplastic left heart syndrome (HLHS), the outcomes in this group of patients have not been determined.
  
  
  METHODS
  We used the Pediatric Heart Transplant Study database (1993 to 2006) to compare outcomes of 388 infants aged < 6 months listed for HLHS, 161 with other congenital heart diseases (non-HLHS), and 145 with cardiomyopathy in early (1993 to 1999) and recent (2000 to 2006) eras.
  
  
  RESULTS
  The cardiomyopathy group was significantly (p < 0.001) different from the HLHS and non-HLHS groups at listing: more girls, older age, and a greater need for high-dose inotropes, mechanical ventilation, and/or mechanical circulatory support. Survival after listing was similar among the groups in the early era. Although outcomes after listing in HLHS and cardiomyopathy patients improved in the recent era, outcomes in non-HLHS patients did not. Survival at 1 and 5 years after listing was significantly worse (p < 0.001) for non-HLHS patients (51%, 48%) vs HLHS (71%, 61%), with age- and sex-adjusted hazard ratio (HR) of 1.79 (95% confidence interval, 1.15-2.77, p = 0.009) and CM (80%, 74%; HR, 2.72; 95% confidence interval, 1.59-4.67, p < 0.001) in the recent era. Post-transplant survival in both eras was not significantly different among the groups.
  
  
  CONCLUSIONS
  Use of heart transplantation as primary therapy for non-HLHS infants has not improved over time and currently is associated with significantly poorer results vs HLHS and cardiomyopathy due to a higher risk for death before transplant.
authors.affiliation: Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: Kristine J
authors.initials: KJ
authors.lastname: Guleserian
authors.forename: Kenneth B
authors.initials: KB
authors.lastname: Schechtman
authors.forename: Jie
authors.initials: J
authors.lastname: Zheng
authors.forename: R Erik
authors.initials: RE
authors.lastname: Edens
authors.forename: Jeffrey P
authors.initials: JP
authors.lastname: Jacobs
authors.forename: William T
authors.initials: WT
authors.lastname: Mahle
authors.forename: Stephanie L
authors.initials: SL
authors.lastname: Emerson
authors.forename: David C
authors.initials: DC
authors.lastname: Naftel
authors.forename: James K
authors.initials: JK
authors.lastname: Kirklin
authors.forename: Elizabeth D
authors.initials: ED
authors.lastname: Blume
authors.forename: Charles E
authors.initials: CE
authors.lastname: Canter
mesh_terms: D009202:Cardiomyopathies / Q000401:mortality / Q000601:surgery*; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D014850:Waiting Lists