2454 Hepatocellular Carcinoma After Fontan Surgery

INTRODUCTION: Tricuspid atresia is a congenital cyanotic heart disease characterized by inability of the blood to flow from the right atrium to the right ventricle. The Fontan operation was first described for tricuspid atresia in 1971. The surgery creates an extra cardiac conduit to maintain circulation in the pulmonary artery without the pump function of the ventricle. This leads to low cardiac output and an increased central venous pressure mostly due to the resistance in the pulmonary circulation. Post- surgery, patients are at risk for liver cirrhosis and possibly hepatocellular carcinoma (HCC), due to venous congestion. Official guidelines do not exist for the screening of patient's for hepatocellular carcinoma after Fontan surgery. We present a case report of a woman with HCC and a history of Fontan surgery. CASE DESCRIPTION/METHODS: A 46-year-old woman with a past medical history of Fontan surgery for tricuspid atresia at age nine presented to the hospital with right upper quadrant discomfort. The patient had a negative history for alcohol abuse, hepatitis or family history for liver disease. Physical examination revealed jaundice and a palpable mass in the right upper quadrant. Laboratory data demonstrated elevated liver enzymes, bilirubin of 1.8 (mg/dl) and an alpha fetoprotein of 1386 (ng/ml). Computed tomographic (CT) scan of the abdomen demonstrated a subcapsular mass with multiple satellite lesions, in the liver. Liver biopsy confirmed moderately differentiated hepatocellular carcinoma. CT scan of lung was compatible with multiple metastatic lesions. She was started on chemotherapy with sorafenib and underwent radio-embolization. She had a poor clinical response to this initial treatment and was subsequently started on nivolumab. The patient was later admitted to the medical intensive care unit with decompensated liver disease and unfortunately, died soon after. DISCUSSION: Her only identifiable risk factor for hepatocellular carcinoma was her history of Fontan surgery. Current opinion is to screen patients for liver disease after Fontan procedure with magnetic resonance imaging (MRI) or CT scan of the liver every 3-5 years, starting at five years after surgery. We aim to increase awareness about the association between Fontan surgery and HCC with the help of our case report.