Fontan Procedure-Associated Hepatocellular Carcinoma: Complications of a Lifesaving Surgery.

Authors

Matthew Pelletier, Huzefa Diwan, Kenney Abraham, Jisung Shin, Ino Chough, Ryan Heslin, Peter Morelli

Published in

JACC. Case reports. Pages 108486. May 22, 2026. Epub May 22, 2026.

Abstract

Fontan surgery is lifesaving for infants with single-ventricle physiology by creating a venocaval bypass tract with the pulmonary arteries; it allows the functioning single ventricle to be used for the systemic circulation. It has been hypothesized that long-standing Fontan circulation increases the risk of cirrhosis and hepatocellular carcinoma (HCC).
A 34-year-old man with tricuspid atresia type 1A after having undergone Fontan surgery 30 years prior presented with a parietal-occipital hematoma after multiple falls, requiring emergent drainage. Computed tomography of the abdomen revealed diffuse metastatic lesions with significantly elevated alpha-fetoprotein levels, concerning for HCC. He was discharged to inpatient hospice care.
The association between Fontan physiology and HCC highlights the importance of early screening of complications to prevent future morbidity and mortality.
This case offers insight on the importance of monitoring for systemic complications of Fontan patients, and it underscores the need for multidisciplinary collaboration and for a standardized screening protocol.

PMID:
42295124
Bibliographic data and abstract were imported from PubMed on 15 Jun 2026.

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