Outcome of Budd-Chiari syndrome: A multivariate analysis of factors related to survival including surgical portosystemic shunting

Abstract

The aim of this study was to assess the factors, including surgical portosystemic shunts, which affect survival in adults with Budd-Chiari syndrome. Multivariate retrospective analysis was performed using characteristics recorded at the time of diagnosis in 120 patients admitted from 1970 to 1992, of whom 82 were treated with surgical portosystemic shunts and 38 received only medical therapy. The 1-, 5-, and 10-year survival rates were 77 ؎ 4%, 64 ؎ 5%, and 57 ؎ 6%, respectively. Survival was significantly better in the subgroup of patients diagnosed after versus before 1985. In both subgroups, and in patients with, as well as in patients without surgical shunts, 4 factors were found to be inversely and independently related to survival: age, response of ascites to diuretics, Pugh score, and serum creatinine. In patients diagnosed since 1985, an index combining these 4 factors allowed to differentiate patients with a good outcome (5-year survival 95%) from those with a poor outcome (5-year survival 62%; P F .05). There was no statistically significant and independent influence of surgical portosystemic shunts on survival. In conclusion, age, severity of liver failure, and presence of refractory ascites are the main prognostic factors in Budd-Chiari syndrome. Increased survival in recent years is consistent with improved management of hypercoagulable states as well as improved general care. It is uncertain whether surgical portosystemic shunting favorably modifies survival. Therefore, we recommend that surgical shunting should be restricted to management of refractory ascites or variceal bleeding in patients with otherwise good prognostic factors. (HEPATOLOGY 1999;30:84-89.)

Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome

“…The first was conducted in France among 120 BCS patients diagnosed between 1970 and 1992. 16 In that study, response of ascites to diuretics, the Child-Pugh score, age, and serum creatinine seemed to be of significant value for the prognosis of BCS. A prognostic index based on these factors dichotomized 85 patients into a good prognostic group (5-year survival, 95%) and a poor prognostic group (5-year survival, 62%), whereas overall 5-year survival was only 65%.…”

“…7,16 This could be because in recent years, improvement in availability and techniques of diagnostic tools has contributed to earlier recognition of BCS patients. 19,24 Furthermore, most of our patients were treated with anticoagulants, which, in addition to better identification of underlying prothrombotic factors, 22 has been reported to contribute to the improvement in prognosis of BCS.…”

“…19,24 Furthermore, most of our patients were treated with anticoagulants, which, in addition to better identification of underlying prothrombotic factors, 22 has been reported to contribute to the improvement in prognosis of BCS. 16 The aim of the present study was to assess prognostic determinants of survival in BCS patients. We identified four important factors that are independently associated with survival: encephalopathy, ascites, prothrombin time, and serum level of bilirubin.…”

“…All participating hospitals serve as tertiary referral centers. Part of the study population from France was described previously by Zeitoun et al 16 and from the Mayo Clinic by Tsiotos et al 22 By means of a standardized review of the medical charts, all patients consecutively diagnosed with BCS between January 1984 and January 2001 were identified using the following key words: Budd-Chiari syndrome, hepatic outflow obstruction, hepatic vein thrombosis, vascular liver disease, hepatic vein, inferior vena cava, portal vein, and thrombosis. BCS was defined as hepatic venous outflow obstruction and its manifestations, regardless of the cause and regardless of the level of the obstruction from the small hepatic veins to the entrance of the inferior vena cava into the right atrium.…”

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

“…In a few examples, medical treatment only was shown to be effective in a subset of patients with stable liver function, diuretic-responsive ascites and asymptomatic presentation [2,67,68] .…”