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The Fontan Liver after Cardiac Transplantation

https://doi.org/10.1016/j.healun.2020.01.1271Get rights and content

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.

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