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Hepatocellular carcinoma diagnosed in a patient who had Fontan operation 30 years ago: a case report
Moon Haeng Hur1, Haeryoung Kim2, Jeong-Hoon Lee1
1Department of Internal Medicine and Liver Research Institute, Seoul National University College of Medicine, Seoul, Korea.
Insights
Patients with a single ventricle undergoing the Fontan operation face risks of liver disease and cancer. Early diagnosis of hepatocellular carcinoma (HCC) through regular surveillance is crucial for long-term outcomes.
Area of Science:
- Cardiology
- Hepatology
- Oncology
Background:
- The Fontan operation, a palliative procedure for single-ventricle defects, can lead to chronic hepatic congestion.
- This congestion is a precursor to Fontan-associated liver disease (FALD), encompassing liver cirrhosis and hepatocellular carcinoma (HCC).
Abstract:
The Fontan operation is performed in patients with a single ventricle. As the systemic venous return is directly connected to the pulmonary circulation during this procedure, chronic hepatic congestion is induced, leading to Fontan-associated liver disease (FALD) including liver cirrhosis and hepatocellular carcinoma (HCC). In this report, we present a case of HCC diagnosed in a patient who underwent the Fontan operation 30 years ago. The patient underwent regular surveillance for FALD, which revealed a 4 cm-sized hepatic mass with elevated serum alpha-fetoprotein. After surgical treatment, there was no evidence of HCC recurrence during 3 years of follow-up. As the risk of HCC and Fontan-associated liver cirrhosis increases with the duration elapsed since the operation, regular surveillance should be emphasized. Serial follow-up of serum alpha-fetoprotein levels and abdominal imaging are necessary to achieve early and accurate diagnosis of HCC in post-Fontan patients.
