Hepatocellular carcinoma in the adult Fontan patient

AbstractIn this study, we describe the case of a 36-year-old woman who was diagnosed with hepatocellular carcinoma on a background of Fontan procedure for tricuspid atresia. She had worsening heart failure in the months before presentation, and early investigations noted derangement in liver enzymes and hepatomegaly. Liver biopsy confirmed a hepatocellular carcinoma. Hepatocellular carcinoma is a rare but recognised consequence of cardiac cirrhosis in Fontan patients.

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Progression of Liver Pathology in Patients Undergoing the Fontan Procedure: Chronic Passive Congestion, Cardiac Cirrhosis, Hepatic Adenoma, and Hepatocellular Carcinoma

Yearbook of Cardiology ◽

10.1016/s0145-4145(07)70070-9 ◽

2006 ◽

Vol 2006 ◽

pp. 122

Author(s):

T.P. Graham

Keyword(s):

Hepatocellular Carcinoma ◽

Fontan Procedure ◽

Liver Pathology ◽

Hepatic Adenoma ◽

Cardiac Cirrhosis ◽

Passive Congestion

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

Seminars in Liver Disease ◽

10.1055/s-0041-1732355 ◽

2021 ◽

Author(s):

Luis Téllez ◽

Enrique Rodríguez de Santiago ◽

Agustín Albillos

Keyword(s):

Hepatocellular Carcinoma ◽

Portal Hypertension ◽

Liver Disease ◽

Fontan Procedure ◽

Elevated Risk ◽

Histological Changes ◽

Global Management ◽

Screening Strategies ◽

History Of ◽

Fontan Patients

AbstractFontan-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.

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Transplant-Amenable Hepatocellular Carcinoma in a Fontan Patient

Case Reports in Gastroenterology ◽

10.1159/000501198 ◽

2019 ◽

Vol 13 (2) ◽

pp. 275-279 ◽

Cited By ~ 2

Author(s):

Brian Patrick Rutledge ◽

Anupama Devara ◽

Sindhuri Benjaram ◽

Stephanie Judd ◽

Murray Ehrinpreis

Keyword(s):

Hepatocellular Carcinoma ◽

At Risk ◽

Liver Transplant ◽

Fontan Procedure ◽

Venous Pressure ◽

Fontan Patient ◽

Number Of Patients ◽

Fontan Physiology ◽

The Ideal

The Fontan circulation alters a patient’s physiology and imparts long-term risks related to chronically elevated systemic venous pressure. An increasing number of patients with Fontan physiology are surviving into adulthood and are at risk of hepatic sequalae. The ideal timeline and method of hepatic surveillance in the Fontan population remains to be defined. In this case, the patient was diagnosed with hepatocellular carcinoma more than 20 years after undergoing the Fontan procedure and was a candidate for combined heart-liver transplant. That her disease progressed prior to organ availability supports the argument for hepatic surveillance in this population.

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