Budd-Chiari syndrome: long-term survival and factors affecting mortality.

Eighteen patients with Budd-Chiari syndrome were studied over a 20 year period to determine the pattern of initial mortality and long-term survival. There were eight early deaths, two due to malignancy, two following surgery, three due to hepatic failure or portal hypertension and one due to septicaemia. The initial high mortality diminished after the first three years and actuarial analysis of survival showed that nearly 50 per cent were alive after five years. The presence of bleeding oesophageal varices indicated poor prognosis. Patients with functioning intrahepatic collaterals seemed to have a longer survival. Many of the surviving patients are leading normal lives with well-controlled symptoms. The prognosis of patients with chronic Budd-Chiari syndrome who survive the initial years of the illness may be better than previously believed.