Hepatocellular carcinoma in Fontan-associated liver disease.

Abstract

Fontan-associated liver disease (FALD) is a significant complication in patients with Fontan palliation. The improved longevity following Fontan palliation has led to wider recognition of FALD and its association with hepatocellular carcinoma (HCC). This review examines the intricate link between FALD and HCC development, emphasizing the unique hemodynamic changes in Fontan circulation that promote hepatic congestion, fibrosis and cirrhosis, thereby facilitating carcinogenesis. The review comprehensively analyzes the existing literature, highlighting key risk factors, pathophysiological mechanisms, and diagnostic challenges in FALD-related HCC. While HCC incidence in FALD remains relatively low (1.5-5.0%), its higher mortality rate of 29.4% necessitates a thorough understanding of contributing factors and screening requirements. The management of FALD involves multidisciplinary approaches, addressing cardiac and hepatic aspects, with regular surveillance for liver disease progression and HCC using advanced imaging and biomarkers. Therapeutic considerations include interventions to manage hepatic congestion and fibrosis, although balancing these with the unique cardiac needs of Fontan circulation remains challenging. Interestingly, FALD management often mirrors that of other liver diseases, underscoring the need for tailored approaches. In severe cases, combined heart-liver transplantation offers a comprehensive solution for FALD-HCC. This review consolidates current knowledge on the epidemiology, pathogenesis and comprehensive management of HCC in the specific context of FALD, ultimately improving outcomes for this unique patient population.