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Spontaneously ruptured hepatocellular carcinoma in Fontan-associated liver disease: A case report
Takahiro Kubo1, Yosuke Aihara1, Hideto Kawaratani1
1Division of Gastroenterology, Third Department of Internal Medicine, Nara Medical University, Nara, Japan.
Insights
The Fontan procedure improves survival for single-ventricle heart defects but increases liver disease risk. This case highlights a patient with Fontan-associated liver disease who developed ruptured hepatocellular carcinoma, emphasizing the need for regular liver monitoring.
Area of Science:
- Cardiology
- Hepatology
- Oncology
Background:
- The Fontan procedure is a critical palliative surgery for single-ventricle physiology, significantly improving long-term survival.
- Increased survival rates have led to a rise in Fontan-associated liver disease (FALD).
Observation:
- A 40-year-old male, status post Fontan procedure at age 9, presented with multiple hepatic tumors.
- Enhanced CT revealed large hepatocellular carcinomas with Vp3 portal vein tumor thrombi.
- The patient experienced spontaneous rupture of hepatocellular carcinoma, necessitating emergent transcatheter arterial embolization.
Findings:
- Autopsy confirmed moderately differentiated hepatocellular carcinoma and a cirrhotic liver with features consistent with FALD (centrilobular fibrosis, sinusoidal dilation).
- This represents the first reported case of spontaneously ruptured hepatocellular carcinoma in the context of FALD.
Implications:
- Early diagnosis of liver cirrhosis and hepatocellular carcinoma in FALD patients is crucial for improved outcomes.
- Cardiologists and hepatologists must maintain a high awareness of FALD and recommend regular liver surveillance for affected patients.
Abstract:
The prognosis of congenital heart disease is dramatically improved by cardiac surgery. The Fontan procedure is the definitive palliative operation for patients with single-ventricle physiology. In parallel with the longer survival time achieved with the Fontan procedure, the incidence of Fontan-associated liver disease is increasing. A 40-year-old man who underwent Fontan procedures at the ages of 9 was referred to our hospital for further evaluation of multiple hepatic tumors. Enhanced computed tomography showed large hepatocellular carcinomas with portal thrombi (Vp3). Spontaneous hepatocellular carcinoma rupture occurred 2 weeks after the first visit to our hospital, and emergent transcatheter arterial embolization of the hepatic artery was performed. Three months later, the patient died of liver failure. Autopsy findings showed moderately differentiated hepatocellular carcinoma with a cirrhotic liver characterized by centrilobular fibrosis and sinusoidal dilation similar to that in Fontan-associated liver disease. We reported the first case of spontaneously ruptured hepatocellular carcinoma treated by emergent transcatheter arterial embolization in Fontan-associated liver disease. As the early diagnosis of liver cirrhosis and hepatocellular carcinoma results in better patients' outcome, cardiologists and hepatologists should be aware of Fontan-associated liver disease and advise patients to have regular follow-up of the liver.
