Fontan-associated liver disease: A review

J Cardiol. 2019 Sep;74(3):223-232. doi: 10.1016/j.jjcc.2019.02.016. Epub 2019 Mar 28.


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.

Keywords: Cirrhosis; Combined heart-liver transplant; Congenital heart disease; Fontan procedure; Hepatocellular carcinoma.

Publication types

  • Review

MeSH terms

  • Carcinoma, Hepatocellular / diagnosis
  • Carcinoma, Hepatocellular / epidemiology*
  • Carcinoma, Hepatocellular / etiology
  • Cardiac Output, Low / epidemiology
  • Cardiac Output, Low / etiology
  • Central Venous Pressure
  • Female
  • Fontan Procedure / adverse effects*
  • Heart Transplantation / methods
  • Humans
  • Incidence
  • Liver Cirrhosis / diagnosis
  • Liver Cirrhosis / epidemiology*
  • Liver Cirrhosis / etiology
  • Liver Function Tests
  • Liver Neoplasms / diagnosis
  • Liver Neoplasms / epidemiology*
  • Liver Neoplasms / etiology
  • Liver Transplantation / methods
  • Male
  • Postoperative Complications / epidemiology*
  • Postoperative Complications / etiology
  • Registries
  • Time Factors
  • Univentricular Heart / surgery*