Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners

Abstract

Nowadays most patients with a univentricular heart after Fontan repair survive until adulthood. One of the hallmarks of Fontan circulation is permanently elevated central venous pressure, which leads to congestive hepatopathy. Subsequently, liver fibrosis, cirrhosis, or hepatocellular carcinoma may occur, all of them constituting an entity called Fontan-associated liver disease (FALD). Given that these complications convey poor prognosis, the need for life-long hepatic surveillance is not in doubt. Many serum biomarkers and sophisticated imaging techniques have been proposed to avoid invasive liver biopsy in this cohort, but none proved to be a relevant surrogate of liver fibrosis seen in histopathological specimens. The surveillance models proposed to date require an extensive diagnostic work-up, which can be problematic, particularly in resource-depleted countries. Moreover, the question of combined heart–liver transplant is gaining more attention in the Fontan cohort. The aim of this study is to provide practical information on the pathophysiology of FALD and to propose a simplified framework for the routine assessment of liver status in Fontan patients that would be helpful in the decision-making process.