Semin Liver Dis 2021; 41(04): 538-550
DOI: 10.1055/s-0041-1732355
Review Article

Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management

1  Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Centro de Investigación Biomédica en Red (CIBEREHD), Universidad de Alcalá, Madrid, Spain
,
Enrique Rodríguez de Santiago
1  Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Centro de Investigación Biomédica en Red (CIBEREHD), Universidad de Alcalá, Madrid, Spain
,
Agustín Albillos
1  Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Centro de Investigación Biomédica en Red (CIBEREHD), Universidad de Alcalá, Madrid, Spain
› Author Affiliations

Abstract

Fontan-associated liver disease is the term used to encompass the disorders arising from abnormal hemodynamic alterations and systemic venous congestion after the Fontan procedure. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is poorly established, many Fontan patients ultimately develop portal hypertension-related complications such as ascites, esophageal varices, malnutrition, and encephalopathy. Fontan survivors also show an elevated risk of hepatocellular carcinoma. Adequate staging of the liver damage is essential to anticipate screening strategies and improve global management.



Publication History

Publication Date:
16 August 2021 (online)

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