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Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes. Int J Cardiol 2021 01 01;322:142-148



Pubmed ID




Scopus ID

2-s2.0-85089963427   5 Citations


BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation.

METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described.

RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months.

CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Author List

Possner M, Gordon-Walker T, Egbe AC, Poterucha JT, Warnes CA, Connolly HM, Ginde S, Clift P, Kogon B, Book WM, Walker N, Wagenaar LJ, Moe T, Oechslin E, Kay WA, Norris M, Dillman JR, Trout AT, Anwar N, Hoskoppal A, Broering DC, Bzeizi K, Veldtman G


Salil Ginde MD, MPH Associate Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Carcinoma, Hepatocellular
Fontan Procedure
Liver Cirrhosis
Liver Neoplasms