Introduction: The Fontan procedure (FP) has become the standard operation for patients with single ventricle physiology. However, a long period of elevated systemic venous pressure and low cardiac output after the procedure result in chronic inflammation and liver cirrhosis, which may eventually lead to the occurrence of hepatocellular carcinoma (HCC).
Clinical findings: We described the case of a 16-year-old female who developed HCC after the FP. At 21 months, the patient received a lateral tunnel FP, and 14 years later, she began complaining of abdominal distension, telangiectasia, and fatigue. Imaging studies revealed a large hepatic mass involving most of the right lobe and multiple masses in the left lobe. Evidence suggested severe liver cirrhosis, and the presence of ascites, hepatosplenomegaly, paraesophageal gastric varices reflecting the severity of the disease. In addition, tumor thrombosis was found in the right hepatic vein, middle hepatic vein, and inferior vena cava, as well as multiple metastatic nodules in both lungs. The patient received an incisional biopsy and the diagnosis of HCC was pathologically confirmed. After treatment with 1 cycle of systemic chemotherapy, she received ongoing supportive care for disease-related complications, and died 2 months after chemotherapy due to hematemesis.
Conclusion: With the advances in medicine, the incidence of Fontan physiology-related complications is likely to increase, and the incidence of HCC will also increase accordingly. As early diagnosis of HCC results in better patient outcomes, a surveillance guideline for HCC after the FP should be developed.