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

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.