title: Outcome of listing for cardiac transplantation for failed Fontan: a multi-institutional study.
abstract: BACKGROUND
  The Fontan procedure is a successful palliation for children with single-ventricle physiology; however, many will eventually require heart transplantation. The purpose of this study was to determine risk factors for death awaiting transplantation and to examine results after transplantation in Fontan patients.
  
  
  RESULTS
  A retrospective, multi-institutional review was performed of 97 Fontan patients <18 years of age listed at 17 Pediatric Heart Transplant Study centers from 1993 to 2001. Mean age at listing was 9.7 years (0.5 to 17.9 years); 25% were <4 years old; 53% were United Network for Organ Sharing status 1; 18% required ventilator support. Pretransplantation survival was 78% at 6 months and 74% at 12 months and was similar to 243 children with other congenital heart disease (CHD) and 747 children without congenital heart disease (No-CHD), who were also awaiting transplantation. Patients who were younger, status 1, had shorter interval since Fontan, or were on a ventilator were more likely to die while waiting. At 6 months, the probability of receiving a transplant was similar for status 1 and 2 (65% versus 68%); however, the probability of death was higher for status 1 (22% versus 5%). Seventy patients underwent transplantation. Survival was 76% at 1 year, 70% at 3 years, and 68% at 5 years, slightly less than CHD and No-CHD patients. Causes of death included infection (30%), graft failure (17%), rejection (13%), sudden death (13%), and graft coronary artery disease (9%). Protein-losing enteropathy (present in 34 patients) resolved in all who survived >30 days after transplantation.
  
  
  CONCLUSIONS
  Heart transplantation is an effective therapy for pediatric patients with a failed Fontan. Although early posttransplantation survival is slightly lower than other patients with CHD, long-term results are encouraging, and protein-losing enteropathy can be expected to resolve.
authors.affiliation: Division of Pediatric Cardiology, Stanford University, 750 Welch Rd, Suite 305, Palo Alto, California 94304, USA. danb@stanford.edu
authors.forename: D
authors.initials: D
authors.lastname: Bernstein
authors.forename: D
authors.initials: D
authors.lastname: Naftel
authors.forename: C
authors.initials: C
authors.lastname: Chin
authors.forename: L J
authors.initials: LJ
authors.lastname: Addonizio
authors.forename: P
authors.initials: P
authors.lastname: Gamberg
authors.forename: E D
authors.initials: ED
authors.lastname: Blume
authors.forename: D
authors.initials: D
authors.lastname: Hsu
authors.forename: C E
authors.initials: CE
authors.lastname: Canter
authors.forename: J K
authors.initials: JK
authors.lastname: Kirklin
authors.forename: W R
authors.initials: WR
authors.lastname: Morrow
mesh_terms: D000293:Adolescent; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D018729:Fontan Procedure; D006331:Heart Diseases / Q000150:complications / Q000151:congenital / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D007223:Infant; D011504:Protein-Losing Enteropathies / Q000209:etiology; D012121:Respiration, Artificial; D012189:Retrospective Studies; D016879:Salvage Therapy / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D015996:Survival Rate; D017211:Treatment Failure; D016896:Treatment Outcome