Budd-Chiari Synthome: Long-term Survival and Factors Affecting Mortality

Eighteen patients with Budd-Chiari syndrome were studied overa 20 year period to determine the pattern of initial mortalityand long-term survival. There were eight early deaths, two dueto malignancy, two following surgery, three due to hepatic failureor portal hypertension and one due to septicaemia. The Initialhigh mortality diminished after the first three years and actuarialanalysis of survival showed that nearly 50 per cent were aliveafter five years. The presence of bleeding oesophageal varicesindicated poor prognosis. Patients with functioning intrahepaticcollaterals seemed to have a longer survival. Mai of the survivingpatients are leading normal lives with well-controlled symptoms.The prognosis of patients with chronic Budd-Chiari syndromewho survive the initial years of the illness may be better thanpreviously believed.