Journal of Liver Cancer

1 "Fontan operation"

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Case Report

Hepatocellular carcinoma diagnosed in a patient who had Fontan operation 30 years ago: a case report: Moon Haeng Hur, Haeryoung Kim, Jeong-Hoon Lee; J Liver Cancer. 2022;22(2):188-193. Published online August 31, 2022; DOI: https://doi.org/10.17998/jlc.2022.08.17

Abstract PDF: 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.

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