Brief Article
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World J Hepatol. Jan 27, 2013; 5(1): 38-42
Published online Jan 27, 2013. doi: 10.4254/wjh.v5.i1.38
Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt
Anders Bay Neumann, Stine Degn Andersen, Dennis Tønner Nielsen, Peter Holland-Fischer, Hendrik Vilstrup, Henning Grønbæk
Anders Bay Neumann, Stine Degn Andersen, Peter Holland-Fischer, Hendrik Vilstrup, Henning Grønbæk, Department of Medicine V (Gastroenterology and Hepatology), Aarhus University Hospital, DK-8000 Aarhus C, Denmark
Dennis Tønner Nielsen, Department of Radiology R, Aarhus University Hospital, DK-8000 Aarhus C, Denmark
Author contributions: Neumann AB, Andersen SD and Grønbæk H designed the study; Andersen SD, Nielsen DT and Holland-Fischer P collected the data and later supplemented by Neumann AB; Neumann AB and Grønbæk H analysed the data; Neumann AB, Holland-Fischer P, Vilstrup H and Grønbæk H wrote the paper; and all authors have approved the final version of the manuscript.
Supported by A grant from the NOVO Nordisk Foundation, to Grønbæk H
Correspondence to: Anders Bay Neumann, MD, Department of Medicine V (Gastroenterology and Hepatology), Aarhus University Hospital, Norrebrogade 44, DK-8000 Aarhus C, Denmark.
Telephone: +45-78462800 Fax: +45-78462860
Received: February 13, 2012
Revised: August 4, 2012
Accepted: December 23, 2012
Published online: January 27, 2013

AIM: To evaluate long-term complications and survival in patients with Budd-Chiari syndrome (BCS) referred to a Danish transjugular intrahepatic portosystemic shunt (TIPS) centre.

METHODS: Twenty-one consecutive patients from 1997-2008 were retrospectively included [15 women and 6 men, median age 40 years (range 17-66 years)]. Eighteen Danish patients came from the 1.8 million catchment population of Aarhus University Hospital and three patients were referred from Scandinavian hospitals. Management consisted of tests for underlying haematological, endocrinological, or hypercoagulative disorders parallel to initiation of specific treatment of BCS.

RESULTS: BCS was mainly caused by thrombophilic (33%) or myeloproliferative (19%) disorders. Forty-three percents had symptoms for less than one week with ascites as the most prevalent finding. Fourteen (67%) were treated with TIPS and 7 (33%) were manageable with treatment of the underlying condition and diuretics. The median follow-up time for the TIPS-treated patients was 50 mo (range 15-117 mo), and none required subsequent liver transplantation. Ascites control was achieved in all TIPS patients with a marked reduction in the dose of diuretics. A total of 14 TIPS revisions were needed, mostly of uncovered stents. Two died during follow-up: One non-TIPS patient worsened after 6 mo and died in relation to transplantation, and one TIPS patient died 4 years after the TIPS-procedure, unrelated to BCS.

CONCLUSION: In our BCS cohort TIPS-treated patients have near-complete survival, reduced need for diuretics and compared to historical data a reduced need for liver transplantation.

Keywords: Ascites, Budd-Chiari syndrome, Myeloproliferative disorder, Thrombophilia, Thrombosis, Transjugular intrahepatic portosystemic shunt