Hepatocellular carcinoma in the adult Fontan patient

Area of Science:

• Cardiology
• Hepatology
• Oncology

Background:

• The Fontan procedure is a palliative surgery for complex congenital heart disease.
• Fontan-associated liver disease (FALD) can lead to cardiac cirrhosis.
• Hepatocellular carcinoma (HCC) is a rare but serious complication of FALD.

Observation:

• A 36-year-old woman with a history of Fontan procedure for tricuspid atresia presented with worsening heart failure.
• Investigations revealed abnormal liver enzymes and hepatomegaly.
• Liver biopsy confirmed hepatocellular carcinoma.

Findings:

• Hepatocellular carcinoma was diagnosed in a patient with a history of Fontan procedure.
• This case underscores the association between cardiac cirrhosis in Fontan patients and HCC development.
• Early detection of liver abnormalities is crucial in this population.

Implications:

• Increased surveillance for HCC may be warranted in adult Fontan survivors.
• Understanding the long-term oncological risks in Fontan patients is critical.
• Further research into the pathogenesis of HCC in Fontan-associated liver disease is needed.