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Rivista di Angiologia
Official Journal of the , the International Union of Phlebology and the
Indexed/Abstracted in: BIOSIS Previews, Current Contents/Clinical Medicine, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
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International Angiology 2016 February;35(1):90-7
Diagnosis, etiology and management of the Budd-Chiari Syndrome: a bloodcoagulation and hepatological study on the course of the disease treated with TIPS
Petr DULÍČEK 1, 2, Petr HŮLEK 3, Antonin KRAJINA 4, Ondřej RENC 4, Václav ŠAFKA 3, Tomas FEJFAR 3, Petr SADÍLEK 1, 2, Martin BERÁNEK 5, Jan J. MICHIELS 6, Pavel ŽÁK 1, 2 ✉
1 Fourth Department of Internal Medicine, Hematology, Faculty of Medicine in Hradec Kralove, University Hospital in Hradec Kralove, Hradec Kralove, Czech Republic; 2 Charles University in Prague, Prague, Czech Republic; 3 Second Department of Internal Medicine, Hematology, Faculty of Medicine in Hradec Kralove, Charles University in Prague, Czech Republic; 4 Department of Interventional Radiology, Faculty of Medicine in Hradec Kralove, Charles University in Prague, Czech Republic; 5 Department of Clinical Biochemistry, Faculty of Medicine in Hradec Kralove, Charles University in Prague, Czech Republic; 6 European International Collaborations and Research on Myeloproliferative Neoplasms: E ICAR. MPN, Goodheart Institute and Foundation in Nature Medicine & Health, Freedom of Science and Education, Rotterdam, The Netherlands
BACKGROUND: Budd-Chiari Syndrome (BCS) is characterized by obstruction of blood flow in hepatic veins. The aim of the study was to analyze diagnosis, etiology and management of BCS.
METHODS: We analyzed 44 patients (32 females, 12 males, the mean age <35y of age) treated with TIPS. Ascites was found in 35 patients as the most frequent symptom. The median of total follow-up was 52 months. Non-covered (bare) or covered stent was inserted to all patients. Diagnosis of myeloproliferative neoplasm (MPN) was based on WHO criteria. Other inherited or acquired thrombophilia were assessed as well. Therapy of BCS was with regard to the etiology.
RESULTS: The etiology of BCS was identified in 38 cases. Ph- MPN was found as the most common risk factor (50%, N.=22), especially polycythemia vera. JAK2V617F mutation was detected in the most of 22 MPN cases (82.5%). The second most common etiologic factor was inherited thrombophilia (18%, N.=8). In the non-covered (bare) stent group, a primary patency rates 52.9% in 1 year and 20% in 5 years after TIPS (Portasystemic Shunt, Transjugular Intrahepatic) creation. In the covered stent group the 1-year and 5-year primary patency rates were was 80% and 33.3% respectively. The average 5-year re-intervention rate per patient was 1.65 procedures in the bare stent group and 0.67 in the covered stent group. Re-interventions were more frequent in MPN patients. All patients were anticoagulated with heparin at the beginning, switched to vitamin K antagonist. On top of TIPS, anticoagulant and a vigorous therapy of underlying disorder are necessary.
CONCLUSION: BCS is a serious and life-threatening disorder in MPD is a major cause of morbidity and mortality. Therapy requires a multidisciplinary approach. Insertion of TIPS dedicated covered stent is a very effective treatment in cases resistant to conservative approach with lower dysfunction rate and the number of re-interventions.