Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome.
Budd-Chiari syndrome (BCS) is a rare disorder that is characterized by hepatic venous outflow obstruction. The aim of this study was to assess determinants of survival and to evaluate the effect of portosystemic shunting. In this international multicenter study, 237 patients with BCS, diagnosed between 1984 and 2001, were investigated. Univariate, multivariate, and time-dependent Cox regression analyses were performed. Overall survival at 1, 5, and 10 years was 82% (95% CI, 77%-87%), 69% (95% CI, 62%-76%), and 62% (95% CI, 54%-70%), respectively. Encephalopathy, ascites, prothrombin time, and bilirubin were independent determinants of survival. A prognostic classification combining these factors could identify three classes of patients (classes I-III). The 5-year survival rate was 89% (95% CI, 79%-99%) for class I, 74% (95% CI, 65%-83%) for class II, and 42% (95% CI, 28%-56%) for class III. Anticoagulants were administered to 72%; only for patients in class I was this associated with a trend toward improved survival (relative risk [RR], 0.14; 95% CI, 0.02-1.21). Portosystemic shunting was performed in 49% of the patients (n = 117); only for patients in class II, time-dependent analyses suggested an improved survival (RR, 0.63; 95% CI, 0.26-1.49). In conclusion, at the time of diagnosis, patients with BCS can be classified into good (I), intermediate (II), and poor (III) prognostic classes, according to simple baseline clinical and laboratory parameters. Our results suggest an improved survival after surgical portosystemic shunting for patients with an intermediate prognosis (class II).
Darwish Murad
Sarwa
S
Department of Gastroenterology and Hepatology, Erasmus Medical Center Rotterdam, Rotterdam, The Netherlands.
Valla
Dominique-Charles
DC
de Groen
Piet C
PC
Zeitoun
Guy
G
Hopmans
Judith A M
JA
Haagsma
Elizabeth B
EB
van Hoek
Bart
B
Hansen
Bettina E
BE
Rosendaal
Frits R
FR
Janssen
Harry L A
HL
D000293:Adolescent; D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D006502:Budd-Chiari Syndrome / Q000188:drug therapy / Q000401:mortality* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011170:Portasystemic Shunt, Surgical / Q000401:mortality*; D011379:Prognosis; D012307:Risk Factors
D000925:Anticoagulants