PURPOSE: Fontan associated liver disease (FALD) is a serious complication of Fontan physiology. In those requiring heart transplant (HT), risk stratification related to liver impairment remains challenging. This multi-center study sought to characterize survival and the natural history of FALD after HT alone. METHODS: Biochemical, imaging and pathologic data were collected retrospectively for all Fontans who underwent HT at 11 pediatric institutions from 2001-2019. Statistical analysis included Wilcoxon signed rank test and univariate Cox proportional hazard regression. Two blinded radiologists reviewed all imaging for consistency. RESULTS: Of 109 transplanted Fontan pts, 56% had decreased ventricular function and 31% had protein losing enteropathy; pts had HT at a median age of 13.7 yrs (IQR 9.5, 17.7), and were 9.9 yrs (4.6, 13.9) out from Fontan operation. Median Fontan pressure pre-HT was 16 mmHg (14, 19) and cardiac index 3 L/min/m2 (2.6, 3.5). Of 100 pts with pre-HT liver imaging (91 ultrasounds, 37 CTs, 18 MRIs), 78% had abnormalities, including nodular contours, hyper enhancing lesions, and fibrotic/cirrhotic changes. Endoscopy noted varices in 5 of 12 pre-HT pts screened, and evidence of sinusoidal dilation/bridging fibrosis in all 12 liver biopsies. Pre-HT lab values were mostly normal and did not differ significantly at 1 yr follow-up except total bilirubin decreased (Table 1). Post-HT imaging in 80 pts frequently showed persistent abnormalities without progression (72%). Eighteen pts died (median time to death from HT 0.6 yrs [0.1, 3.4]) with 2 deaths possibly related to FALD; 1 pt suffered a GI bleed 6 wks after HT, while a second died at 2 months with portal hypertension and pulmonary AVMs. No abnormal lab values, cath hemodynamics or imaging were associated with death. CONCLUSION: FALD at the time of HT was not a significant cause of early mortality in this cohort. Abnormal liver features persisted on early post HT follow-up imaging without progression of disease or associated lab aberrations indicating HT may improve long term prognosis.
