Abstract 9684: Favourable Midterm Outcome After Heart Transplantation for Late Fontan Failure

Abstract

Background: Fontan failure (FF) represents a growing and challenging indication for pediatric orthotopic heart transplantation (OHT).The aim of this study was to identify predictors of best midterm outcome in OHT after FF. Methods: retrospective multi-institutional study on 45 consecutive patients who underwent OHT for FF between 1992 ans 2011. Mean follow-up was 72.8±70.5 month and was 100% complete. Early FF was defined as failure requiring OHT within 2 years from Fontan completion. Results: 45 patients underwent OHT 8.6±6.1 years after Fontan completion due to failing atriopulmonary (12) or TCPC (33). Modality of FF included protein losing enteropathy (PLE) (31%), complex obstructions in Fontan circuit (22%), arrhythmia (18%) and impaired ventricular function (29%). Early FF occurred in 15.6% patients. Mean age at OHT was 12.4±4.9 years. Hospital mortality was 17.8% and late mortality was 13.5%. Main causes of early death were infection (37.5%) and graft failure (37.5%). Mean time of mechanical ventilation was 8.6±2.3 days while mean hospital stay was 40.1±7.2 days. Overall Kaplan Meier (KM) survival was 82.2±5.7% at 1 year, 78.4±7.0% at 5 years and 55.3±11.2% at 10 years. KM one-year OHT survival was 89.5±5.1% in late FF and 42.9±9.7% in early FF (p=0.0031). Fisher exact testing showed a strong correlation between late FF and impaired ventricular function (p=0.0226). OHT survival for late FF was 84.5±6.7% at 5 years and 59.7±13.0% at 10 yrs. Protein dispersion improved after OHT in 78.6% patients with PLE, but overall 5-year OHT survival in PLE was 45.9±12.1 versus 87.1± 6.0% in non-PLE (p=0.027). Early FF (p=0.0017), PLE (p=0.0157) and complex obstructions of Fontan circuit (p=0.0087) were identified as independent predictors of mortality by Cox proportional hazards. Conclusions: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE and complex obstructions of Fontan circuit negatively affect midterm OHT outcome.