Fontan-associated liver disease: A review

•The Fontan procedure for single ventricle pathology has improved survival into adulthood.•Fontan-associated liver disease (FALD) is a major non-cardiac determinant of mortality following the Fontan procedure.•The incidence of liver cirrhosis and hepatocellular carcinoma (HCC) increases with the duration of the Fontan circulation.•The diagnosis of FALD requires careful review of clinical features, biochemical/hematological parameters, imaging, non-invasive fibrosis scores, and (if necessary) liver histology.•Combined heart-liver transplant may be required for FALD complicated by liver cirrhosis or HCC. The Fontan procedure has led to increased long-term survival of patients with single ventricle congenital heart disease. Hemodynamic changes associated with the Fontan circulation, including elevated central venous pressure and diminished cardiac output are responsible for the development of Fontan-associated liver disease (FALD). Liver fibrosis is a universal feature following the Fontan operation. The incidence of both liver cirrhosis and hepatocellular carcinoma (HCC) increases with the duration of the Fontan circulation. The staging of liver fibrosis in FALD requires a multi-modality approach involving clinical assessment, biochemical/hematological parameters, non-invasive fibrosis scores, radiological imaging, elastography, and liver histology. Patients with a failing Fontan circulation who have evidence of significant hepatic congestion require careful hemodynamic assessment to optimize the Fontan pathway and physiology. This may necessitate percutaneous or surgical intervention, or heart transplantation. Combined heart-liver transplantation may be required in patients with clinical, imaging, or biopsy evidence of advanced liver cirrhosis, particularly if there is evidence of hepatic decompensation or localized HCC. Patients with suspected liver cirrhosis should be enrolled into HCC surveillance and require endoscopic variceal assessment. There is a clear need to establish local/national registries for Fontan patients with standardized guidelines for the management of FALD, bringing together the expertise of professional bodies representing both cardiologists and hepatologists.