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

Luis Téllez; Enrique Rodríguez de Santiago; Agustín Albillos

doi:10.1055/s-0041-1732355

Seminars in Liver Disease, Inhaltsverzeichnis

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

Review Article

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

Luis Téllez

¹Department 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

¹Department 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

¹Department 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

› Institutsangaben

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.

Keywords

Fontan-associated liver disease - Fontan surgery - cirrhosis - hepatocellular carcinoma

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Referenzen

References
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