Abstract 16359: Incidence of Fontan Associated Liver Disease and Its Impact on Mortality in Patients With Single Ventricle Physiology

Abstract

Introduction: Data on the burden of clinically significant Fontan associated liver disease (FALD) and its relationship to mortality is scant. We performed a retrospective cohort study to assess the incidence of FALD and its association with mortality. Methods: Data source was the Quebec Congenital Heart Disease (CHD) Database, a population-based cohort of over 100,000 CHD patients followed from 1983-2017. Fontan patients surviving longer than 30 days post-Fontan were identified, each were matched to 20 VSD patients on age and sex. The Fontan-VSD cohort were used to assess the association between Fontan and the risk of developing FALD. The VSD cohort served as “Non-exposed to Fontan” group. FALD was defined as at least one hospitalization due to liver disease. Kaplan-Meier curves were used to estimate and compare the cumulative probability of 1) developing FALD between Fontan and VSD patients; and 2) mortality between Fontan patients w/o FALD. Results: A total of 512 Fontan patients and 10,232 VSD patients were included. The cumulative probability of developing FALD at 10 and 25 years of follow-up was higher in Fontan patients (13.0% and 37.1%, respectively), compared to 0.7% and 2.0% for VSD patients respectively (p-value <0.0001-Logrank test). In Fontan patients with FALD, the cumulative probability of mortality by 5 years after the diagnosis of FALD was 12.6%, 11 times higher than the risk in Fontan Patients without FALD (Figure) . Calendar year of Fontan operation was found to be an important predictor of developing FALD. Conclusions: This is the first study documenting the impact of FALD on mortality, using a large cohort with long-term follow up. The findings support the use of well-defined surveillance protocols to identify potential precipitants of FALD before liver disease becomes irreversible.