long-term outcome of budd-chiari syndrome:a single center experience

background budd-chiari syndrome (bcs) is defined as hepatic venous outflow obstruction (hvoo). bcs is an uncommon, life-threatening liver disorder. this study describes the clinical and etiological characteristics in addition to the long-term outcome of bcs in a single referral center in tehran, iran. methods we reviewed long-term outcome of patients who were diagnosed with bcs between 1989 and 2012 at shariati hospital, a tertiary hospital affiliated with tehran university of medical sciences, tehran, iran. the diagnosis was confirmed by at least two imaging techniques. a comprehensive analysis of the clinical and paraclinical manifestations, etiology and long-term outcome of the disease was conducted. results during the 22-year study period we identified 71 bcs patients, 28 (39%) males and 43 (60.1%) females, who ranged in age from 17 to 64 years (median: 29 years). we excluded 16 patients because of incomplete information or follow up. the remaining 55 cases were the subjects of this study. underlying etiologies consisted of congenital thrombophilia factors in 50% (28 cases) which was defined as protein c deficiency (12 cases), protein s deficiency (3 cases), antithrombin deficiency (3 cases) and factor v leiden mutation (10 cases). etiology was unknown in 18% (10 cases). acquired causes of thrombophilia were observed in 25% (14 cases) that consisted of 9 cases of myeloproliferative disease and 5 cases of autoimmune diseases. in 3 cases pregnancy was the only etiology. the main clinical presentations were abdominal pain in 33 (60%), abdominal distention in 21 (38.2%), and jaundice in 10 (18%) cases. the main clinical signs were ascites (76.4%), splenomegaly (34%), hepatomegaly (25.5%) and deep vein thrombosis (1.8%). all 55 patients were treated with anticoagulants (heparin followed by warfarin) and supportive care. two cases underwent mesocaval shunting, 2 patients required transjugular portosystemic shunting (tips) and 5 were referred for liver transplantation. a total of 17 (30%) patients died during 22 years of follow up. conclusion bcs, although uncommon in iran, is a challenging liver disease with an important burden. medical therapy that includes anticoagulation seems to be effective in most cases although the prognosis is guarded. in long-term follow up, 40% of cases will need liver transplant or die from end stage liver disease.