Spontaneously ruptured hepatocellular carcinoma in Fontan-associated liver disease: A case report


Kubo T, Aihara Y, Kawaratani H, Namisaki T, Noguchi R, Mitoro A, Yoshiji H.SAGE Open Med Case Rep. 2020 May 6;8:2050313X20922030. doi: 10.1177/2050313X20922030. eCollection 2020.PMID: 32477565 Free PMC article.



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.


Figure 1. Enhanced CT imaging at the patient’s first visit to our hospital. The results of CT showed a large hepatic tumor that occupied the liver, with left portal invasion (arrowhead) and metastasis of the left adrenal gland (arrow).


Figure 2. Enhanced CT imaging and angiography at the time of HCC rupture. (a) The results of enhanced CT showed intraperitoneal ascites and high-density fluid pooled around the HCC within strange vessels in segment 3 (arrow). (b) The results of emergent angiography showed multiple hepatic tumor stains and irregular vessels in segment 3 (arrowhead).


Figure 3. Macroscopic examination of the liver at autopsy. The liver was swollen and almost completely occupied by multiple tumors.


Figure 4. Histological examination of the liver tumor and non-cancerous lesions at autopsy. (a) Countless coated nodules occupied the liver (HE staining, 10-fold). (b) The moderately differentiated HCC showed a thick trabecular pattern, stratified appearance, and false duct structures of the cord-like type (HE staining, 100-fold). (c) Fibrosis around the central vein and developing bridging fibrosis were observed (Azan staining, 10-fold). (d) Sinusoidal dilation was apparent around the central vein. Interface hepatitis, hemorrhage, necrosis, and steatosis were not observed (HE staining, 40-fold).