Favourable mid-term outcome after heart transplantation for late Fontan failure

• Published in: European journal of cardio-thoracic surgery 2015-04, Vol.47 (4), p.665-671
• Main Authors: Michielon, Guido, van Melle, Joost P., Wolff, Djoeke, Di Carlo, Duccio, Jacobs, Jeffrey P., Mattila, Ilkka P., Berggren, Hakan, Lindberg, Harald, Padalino, Massimo A., Meyns, Bart, Prêtre, René, Helvind, Morten, Carrel, Thierry, Ebels, Tjark
• Format: Article
• Language: English
• Subjects: Adolescent; Child; Child, Preschool; Female; Fontan Procedure - adverse effects; Fontan Procedure - statistics & numerical data; Heart Defects, Congenital - epidemiology; Heart Defects, Congenital - surgery; Heart Failure; Heart Transplantation - mortality; Humans; Infant; Kaplan-Meier Estimate; Male; Retrospective Studies; Treatment Outcome
• ISSN: 1010-7940; 1873-734X
• DOI: 10.1093/ejcts/ezu280

OBJECTIVES Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan–Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.