Outcomes after transplantation for "failed" Fontan: a single-institution experience

J Thorac Cardiovasc Surg. 2012 May;143(5):1183-1192.e4. doi: 10.1016/j.jtcvs.2011.12.039.


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.

MeSH terms

  • Adolescent
  • Adult
  • Chi-Square Distribution
  • Child
  • Child, Preschool
  • Female
  • Fontan Procedure / adverse effects*
  • Fontan Procedure / mortality
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / surgery*
  • Heart Transplantation* / adverse effects
  • Heart Transplantation* / mortality
  • Heart Ventricles / abnormalities
  • Heart Ventricles / physiopathology
  • Heart Ventricles / surgery*
  • Hemodynamics
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Multivariate Analysis
  • New York City
  • Odds Ratio
  • Patient Selection
  • Reoperation
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Treatment Failure
  • Young Adult