title: Outcomes after transplantation for "failed" Fontan: a single-institution experience.
abstract: OBJECTIVE
  Despite the excellent outcomes in the current era after the Fontan procedure, it continues to have an inherent risk of failure. Cardiac transplantation provides 1 option for treating these patients; however, the indications for, timing of, and outcomes after, transplantation remain undefined. We examined our own institutional experience with transplantation for failed Fontan.
  
  
  METHODS
  The records of 155 patients transplanted for congenital heart disease at a single institution from June 1984 to September 2007 were reviewed. Of these patients, 43 had undergone a previous Fontan procedure (25 male, 15 female; median age, 14.5 years; range, 1-47; 23 classic Fontan, 13 lateral tunnel, 4 extracardiac conduit, and 3 revised to shunt). The predictors of short- and long-term survival were evaluated, and the Fontan patients were compared with all other patients with congenital heart disease (n = 129, 78 male, 51 female).
  
  
  RESULTS
  The most common indications for transplantation included protein-losing enteropathy (PLE) (39.5%), chronic heart failure (41.8%), and acute post-Fontan failure (9.3%). The transplants performed in Fontan patients were more likely to require pulmonary artery reconstruction (85.4% vs 42.9%; P < .0001) and had longer cardiopulmonary bypass times (278 vs 179 minutes; P < .0001). The 90-day mortality rate was greater in the Fontan group (35.0% vs 20.0%; P = .055). No correlation was observed between the interval from Fontan to transplantation and morality; however, renal failure was a strong predictor of early mortality (odds ratio, 10.8; 95% confidence interval, 1.5-75.7).
  
  
  CONCLUSIONS
  Transplantation is an acceptable treatment for patients with a failed Fontan. Clinical factors (instead of the indication for transplantation) appear to have the greatest correlation with early mortality.
authors.affiliation: Nemours Cardiac Center, A.I. duPont Hospital for Children, 1600 Rockland Rd, Wilmington, DE 19803, USA. rdavies@nemours.org
authors.forename: Ryan R
authors.initials: RR
authors.lastname: Davies
authors.forename: Robert A
authors.initials: RA
authors.lastname: Sorabella
authors.forename: Jonathan
authors.initials: J
authors.lastname: Yang
authors.forename: Ralph S
authors.initials: RS
authors.lastname: Mosca
authors.forename: Jonathan M
authors.initials: JM
authors.lastname: Chen
authors.forename: Jan M
authors.initials: JM
authors.lastname: Quaegebeur
mesh_terms: D000293:Adolescent; D000328:Adult; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009519:New York City; D016017:Odds Ratio; D018579:Patient Selection; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D017211:Treatment Failure; D055815:Young Adult