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

Background: 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). Case Summary: 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. Discussion: The association between Fontan physiology and HCC highlights the importance of early screening of complications to prevent future morbidity and mortality. Take-Home Message: 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.