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

Figure

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  Figure 1. Effect of Fontan operation on the development of Fontan-associated liver disease. In Fontan circulation, the systemic veins and pulmonary arteries are directly connected. Elevated central venous pressure induced by passive filling of the pulmonary circulation without the support of the right ventricle results in chronic hepatic congestion. In addition, low cardiac output due to limited preload reduces portal blood flow, which contributes to ischemic insults. Consequently, accumulated liver damage can lead to liver cirrhosis and even hepatocellular carcinoma.

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  Figure 2. Computed tomography image showing a 4 cm-sized hepatic mass in segment V (arrows). The mass is seen as hyperenhancement in the arterial phase (A) and washout in the portal venous phase (B).

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  Figure 3. Contrast-enhanced liver magnetic resonance imaging showing a well-circumscribed mass (arrow) with arterial phase hyperenhancement (A) and hepatobiliary phase defect (arrow) (B). In a T2-weighted image, it shows a high signal intensity (arrow) (C). The hepatic mass exhibited significant hypermetabolism in the positron emission tomography-computed tomography image (arrow) (D).

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  Figure 4. On histological examination, a moderately differentiated hepatocellular carcinoma with a trabecular pattern was observed (A, hematoxylin and eosin stain, ×200). The background liver shows micronodular venocentric cirrhosis (B, Masson’s trichrome stain, ×100).

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