SHM Converge Scientific Abstract ePoster Gallery-Metastatic Hepatocellular Carcinoma After Fontan Procedure: A Can't-Miss Complication of Patients With Congenital Heart Disease

Metastatic Hepatocellular Carcinoma After Fontan Procedure: A Can't-Miss Complication of Patients With Congenital Heart Disease

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This case describes a 33-year-old man with a history of hypoplastic left heart syndrome, status post Fontan procedure at age 3, who presented with 5 days of lower back pain. Initial outpatient CT imaging showed cirrhotic liver changes and multiple enhancing hepatic lesions, and further evaluation with CT pelvis and MRI spine revealed metastatic bone lesions involving L3 and both iliac wings, with severe spinal canal stenosis. Laboratory studies showed elevated INR, alkaline phosphatase, and markedly elevated AFP (7256), raising strong concern for hepatocellular carcinoma.<br /><br />He was admitted to the med-peds service, underwent CT-guided biopsy of the left iliac wing, and was discharged home once pain was controlled with oncology follow-up. Pathology confirmed poorly differentiated hepatocellular carcinoma. After discussion of treatment options, the family chose palliative radiation and a focus on symptom control.<br /><br />The key teaching point is that Fontan circulation alters hemodynamics and can cause chronic hepatic venous congestion, which may progress to cirrhosis and hepatocellular carcinoma. In patients with congenital heart disease who have undergone Fontan repair, HCC is a “can’t-miss” complication. The mean age at HCC diagnosis after Fontan is about 30 years, and current guidance recommends liver function tests and AFP screening every 1–2 years after Fontan.<br /><br />As more patients with congenital heart disease survive into adulthood, hospitalists and other clinicians are increasingly likely to encounter this population. Awareness of Fontan-associated liver disease and its cancer risk is essential for timely diagnosis and management.

Asset Subtitle

Jesse Rhodes

Meta Tag

Author List Jesse Rhodes

Category Clinical Vignettes

Concept Fontan Procedure

Concept Hepatocellular Carcinoma

Concept Venous Congestion

Concept Cirrhosis

Concept Alpha-Fetoprotein

Distinguished Non-Finalist

Presenter Organization Duke University Hospital

Presenting Author Jesse Rhodes

Track Adult

Keywords

Fontan procedure

hypoplastic left heart syndrome

hepatocellular carcinoma

cirrhosis

hepatic lesions

metastatic bone lesions

alpha-fetoprotein

Fontan-associated liver disease

congenital heart disease

AFP screening

Fontan Procedure

Hepatocellular Carcinoma

Venous Congestion

Cirrhosis

Alpha-Fetoprotein