Juvenile hepatocellular carcinoma with congestive liver cirrhosis

Abstract

A case of juvenile hepatocellular carcinoma (HCC) with congestive liver cirrhosis is reported. The patient was a 21-year-old woman. She had been diagnosed as having transposition of the great arteries, type 2, in 1978. She underwent the Mustard operation, but suffered from chronic heart failure. In 1995, she experienced abdominal pain and underwent examination. The laboratory data were normal, except for elevated total bilirubin (5.2 mg/dl). Blood examinations were performed at frequent intervals, and the total bilirubin level fluctuated between 0.9 and 8.1 mg/dl over the next 4 years, but the transaminase level remained normal. In 1999, she experienced abdominal pain again and was admitted to our hospital. Computed tomography showed four space-occupying lesions in the liver; 45 mm, 20 mm, 12 mm, and 10 mm in size. She was diagnosed as having HCC, and transcatheter arterial chemoembolization and percutaneous ethanol injection therapy were performed. Histology of the cancerous and the noncancerous liver tissue revealed HCC, moderately differentiated type, in cirrhotic liver with congestion. This patient had no background factors of liver disease, except for liver congestion, associated with the chronic heart failure. Because most patients with cardiac cirrhosis die of cardiac disease, only a small number of these patients develop liver failure. However, the incidence of HCC in patients with congestive liver disease is likely to increase in the future, as survival time is prolonged with the advances in treatment for chronic heart failure. Therefore, patients with congestive liver disease should be followed, taking into account the possibility of HCC.

Cardiac cirrhosis and hepatocellular carcinoma in a 13‐year‐old treated with doxorubicin microbead transarterial chemoembolization

“…Two of these followed long‐standing venous congestion because of adult acquired restrictive peri‐carditis 1,2 . The other four occurred in patients with congenital cardiac defects treated with Fontan or Mustard operation 3–5 . Their age of presentation with HCC was in the third and fourth decade of life unlike our patient who developed HCC aged 13 years.…”

Arterialised hepatic nodules in the Fontan circulation: Hepatico-cardiac interactions

“…All 3 demonstrated benign features with SPIO uptake on MRI in 2 patients and no late lipiodol retention on CT in the other patient. (16)(17)(18). Compared to those without, patients with nodules had higher mean RA pressures (18 mmHg ±5.6 vs. 13 mmHg ±4, p=0.025), where as their mixed venous saturation and aortic saturation was not significantly different (70%±11 vs. 67%±9 and 92%±10 vs. 94%±4, pN 0.05).…”

“…These findings resemble nodules documented in other chronic venous outflow conditions like Budd Chiari syndrome. [15] Despite these reassuring features there are reported cases of hepatocellular carcinoma in Fontan patients with cardiac hepatopathy [2,[16][17][18].…”

Does pressure cause liver cirrhosis? The sinusoidal pressure hypothesis

“…Cardiac cirrhosis can develop rapidly even in young patients with congenital malformation of the heart after the Fontan procedure. These patients will develop portal hypertension, esophageal varices and they can even die from primary liver cancer[49,50]. Figure 6 shows a typical finding of bridging cirrhosis both in an elderly patient with chronic heart failure (Figure 6A-F) and in a young patient after Fontan operation (Figure 6G and H).…”