*Purpose: To add to the clinical characteristics and outcomes of hepatocellular carcinoma (HCC) in post Fontan operation patients and present, to the best of our knowledge, the largest single center case series.

*Methods: In this single center retrospective study, medical records were obtained from patients who developed HCC after Fontan palliation. Charts were reviewed for identifying characteristics, cardiac history, laboratory data, features of portal hypertension, imaging, type of therapy, and outcomes.

*Results: Nine cases of HCC following Fontan palliation were identified. The majority of subjects were male and Caucasian with average age at time of Fontan 5.4 years and 27.5 at diagnosis of HCC. One patient had co-existing liver disease with HCV. None had significant EtOH history. Mean BMI was 25.5. Type of congenital heart disease varied, with three patients having tricuspid atresia and two, ultimately, requiring Fontan revision. Right heart catheterization (RHC) was performed in four patients with evidence of elevated Fontan pressures in all. Median VAST score (Varices, Ascites, Splenomegaly, Thrombocytopenia) was 2. Most common presentation of HCC was with routine imaging compared to elevated AFP and suspicion for metastatic mass. Imaging at time of HCC was largely MRI compared to CT (8 vs 2). Mean diameter of HCC was 1.65 cm with one patient having multiple arterial enhancing lesions. 2 patients had metastases at time of diagnosis. 5 of 9 had normal AFP levels at time of diagnosis. Therapy involved CT guided microwave ablation, Y90 radioembolization, and chemotherapy/radiation in 1 patient with metastatic disease. 5 subjects had no evidence of recurrence after therapy while others had either recurrence, new lesion, or metastases. None had a liver transplant. At time of study, 7 patients were undergoing surveillance with repeat imaging while the two patients with metastatic disease expired.

*Conclusions: The Fontan operation for congenital heart disease is associated with chronically elevated central venous pressure and cardiac induced liver disease. HCC can occur after Fontan palliation, most often in early adulthood. Average time from procedure to diagnosis of HCC was 22.1 years. Diagnosis can be made with routine screening imaging for HCC and/or elevated AFP, which may not necessarily be elevated at time of diagnosis. The diagnosis is associated with features of portal hypertension. A minority of patients expired from metastatic disease while most had relatively successful clinical courses. Overall, these patient’s cardiac disease appears to limits their candidacy for a liver transplant.

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