title: Fontan Patient Survival After Pediatric Heart Transplantation Has Improved in the Current Era.
abstract: BACKGROUND
  Historically, patients with a prior Fontan procedure for complex congenital heart disease (CHD) have been considered at higher risk for death after heart transplant (HT) compared with other HT transplant candidates. With the overall trend of improved survival of pediatric HT recipients, it is unclear of Fontan patient post-HT survival has also improved in the current era.
  
  
  METHODS
  Data from the Pediatric Heart Transplant Study database for Fontan patients who underwent HT was compared between the early era (1993 to 2006, n = 150) and late era (2007 to 2014, n = 252). Post-HT survival and pre-HT characteristics were compared among eras and also with non-Fontan CHD patients.
  
  
  RESULTS
  At time of HT, Fontan patients in the late era were more likely to require inotropic support, have protein-losing enteropathy, have failure to thrive, and be further from time of Fontan, although less likely to be on ventilator support. Only ventilator support and earlier year of HT were significant risk factors for death in the multivariate analysis. Post-HT Fontan patient survival significantly improved from the early to late era (p = 0.02), particularly in the early phase, with 1-year survival of 77% in the early era and 89% in the late era. Late era non-Fontan CHD patient 1-year post-HT survival was similar to Fontan patients at 92%.
  
  
  CONCLUSIONS
  Survival of Fontan patients after HT has significantly improved in the current era. Currently, expected post-HT survival for Fontan patients is on par with other CHD patients. Fontan patients should not be excluded from consideration for HT solely on a history of Fontan.
authors.affiliation: Department of Pediatrics, St. Louis Children's Hospital, St. Louis, Missouri. Electronic address: simpson_k@kids.wustl.edu.
authors.forename: Kathleen E
authors.initials: KE
authors.lastname: Simpson
authors.affiliation: Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Pruitt
authors.affiliation: Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: James K
authors.initials: JK
authors.lastname: Kirklin
authors.affiliation: Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: David C
authors.initials: DC
authors.lastname: Naftel
authors.affiliation: Department of Pediatrics, University of California San Diego, Rady Children's Hospital, San Diego, California.
authors.forename: Rakesh K
authors.initials: RK
authors.lastname: Singh
authors.affiliation: Department of Pediatrics, University of Iowa Children's Hospital, Iowa City, Iowa.
authors.forename: R Erik
authors.initials: RE
authors.lastname: Edens
authors.affiliation: Department of Pediatrics, Children's Mercy Hospital, Kansas City, Missouri.
authors.forename: Aliessa P
authors.initials: AP
authors.lastname: Barnes
authors.affiliation: Department of Pediatrics, St. Louis Children's Hospital, St. Louis, Missouri.
authors.forename: Charles E
authors.initials: CE
authors.lastname: Canter
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality* / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology*; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome