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.
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: Jacob R Miller; Kathleen E Simpson; Deirdre J Epstein; Timothy S Lancaster; Matthew C Henn; Richard B Schuessler; David T Balzer; Shabana Shahanavaz; Joshua J Murphy; Charles E Canter; Pirooz Eghtesady; Umar S Boston Journal: J Heart Lung Transplant Date: 2016-03-10 Impact factor: 10.247
Authors: Lindsay R Freud; Doff B McElhinney; Audrey C Marshall; Gerald R Marx; Kevin G Friedman; Pedro J del Nido; Sitaram M Emani; Terra Lafranchi; Virginia Silva; Louise E Wilkins-Haug; Carol B Benson; James E Lock; Wayne Tworetzky Journal: Circulation Date: 2014-07-22 Impact factor: 29.690
Authors: Jill M Gelow; Howard K Song; Joseph B Weiss; James O Mudd; Craig S Broberg Journal: J Heart Lung Transplant Date: 2013-08-06 Impact factor: 10.247
Authors: Brian W McCrindle; Victor Zak; Roger E Breitbart; Lynn Mahony; Peter Shrader; Wyman W Lai; Kristin M Burns; Steven D Colan; Richard V Williams; David Goldberg; Kevin D Hill; Svetlana Khaikin; Andrew M Atz Journal: Pediatr Cardiol Date: 2013-11-22 Impact factor: 1.655