Spontaneous rupture of hepatocellular carcinoma in Fontan-associated liver disease with fatal outcome: case report and literature review.

The Fontan procedure is the definitive palliative operation for patients with single-ventricle physiology. In parallel with the longer survival time achieved by the Fontan procedure, however, the incidence of Fontan-associated liver disease is increasing as well. A 25-year-old man who underwent the Fontan procedure at the age of 3 years was referred to our hospital for further evaluation of congestive hepatopathy under suspicion of Fontan-associated liver disease. Upon presentation, a heterogeneous mass of 32 × 27 mm was identified in the S4 region of the liver. The tumor showed progressive enlargement along with a rising α-fetoprotein level. We suspected hepatocellular carcinoma and scheduled a hepatectomy. On the first day of hospitalization for preoperative examination in our cardiology department, he unexpectedly suffered spontaneous hepatocellular carcinoma rupture. In spite of emergency transcatheter arterial embolization of the hepatic artery and additional interventions, the patient ultimately succumbed to intraperitoneal bleeding. Given the poor prognosis of HCC rupture in Fontan-associated liver disease, early intervention is crucial to prevent fatal outcomes.