A 34-year-old man developed metastatic hepatocellular carcinoma 30 years after undergoing Fontan surgery for tricuspid atresia type 1A, highlighting the need for systemic complication screening.
Case Report (n=1)
Does long-standing Fontan circulation increase the risk of hepatocellular carcinoma?
Long-standing Fontan circulation may be associated with the development of hepatocellular carcinoma, highlighting the need for standardized screening protocols.
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.
Pelletier et al. (Mon,) conducted a case report in Tricuspid atresia type 1A / Hepatocellular carcinoma (n=1). Fontan surgery was evaluated on Hepatocellular carcinoma. A 34-year-old man developed metastatic hepatocellular carcinoma 30 years after undergoing Fontan surgery for tricuspid atresia type 1A, highlighting the need for systemic complication screening.
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