J Liver Cancer.  2022 Sep;22(2):188-193. 10.17998/jlc.2022.08.17.

Hepatocellular carcinoma diagnosed in a patient who had Fontan operation 30 years ago: a case report

• Hur MH
• Kim H
• Lee JH

• KMID: 2534246
• DOI: http://doi.org/10.17998/jlc.2022.08.17

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.

Keyword

Liver cancer; Fontan operation; Fontan-associated liver disease; Case report