Liver transplantation for Budd-Chiari syndrome: A European study on 248 patients from 51 centres.
OBJECTIVE
The results of liver transplantation for Budd-Chiari syndrome (BCS) are poorly known and the role and timing of the procedure are still controversial. The aim of this study was to investigate the results of transplantation for BCS, focusing on overall outcome, on prognostic factors and on the impact of the underlying disease.


METHODS
An enquiry on 248 patients representing 84% of the patients transplanted for BCS in the European Liver Transplantation Registry between 1988 and 1999.


RESULTS
Of the 248 patients, 70.4% were female and 29.6% male. The mean age was 35.7 years. The overall actuarial survival was 76% at 1 year, 71% at 5 years and 68% at 10 years. 77% of deaths occurred in the first 3 months: 47% were due to infection and multiple organ failure, and 18% to graft failure or hepatic artery thrombosis. Late mortality (>1 year) occurred in nine patients, due to BCS recurrence in four of them. The only pre-transplant predictors of mortality on multivariate analysis (Cox) were impaired renal function and a history of a shunt.


CONCLUSIONS
Liver transplantation for BCS is an effective treatment, irrespective of the underlying cause, and should be considered before renal failure occurs.
Mentha
Gilles
G
Transplantation Unit, Department of Surgery, University Hospitals, 1211 Geneva 14, Switzerland. gilles.mentha@hcuge.ch
Giostra
Emiliano
E
Majno
Pietro E
PE
Bechstein
Wolf O
WO
Neuhaus
Peter
P
O'Grady
John
J
Praseedom
Raaj K
RK
Burroughs
Andrew K
AK
Le Treut
Yves P
YP
Kirkegaard
Preben
P
Rogiers
Xavier
X
Ericzon
Bo-Goran
BG
Hockerstedt
Krister
K
Adam
René
R
Klempnauer
Juergen
J
D000328:Adult; D001706:Biopsy; D006502:Budd-Chiari Syndrome / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D005060:Europe / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006801:Humans; D016031:Liver Transplantation / Q000401:mortality / Q000706:statistics & numerical data*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors