Budd-Chiari Syndrome: Anticoagulation, TIPS, or Transplant
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PORTAL HYPERTENSION (J GONZALEZ-ABRALDES AND E TSOCHATZIS, SECTION EDITORS)
Budd-Chiari Syndrome: Anticoagulation, TIPS, or Transplant Marta Magaz 1,2,3 & Guillem Soy 1,2,3 & Juan Carlos García-Pagán 1,2,3
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Background and Purpose of Review Budd-Chiari syndrome (BCS) is a rare vascular liver disease caused by the obstruction of the hepatic venous outflow tract that may be fatal and is not adequately treated. We aim to review the current management of BCS. Methods Analysis of recent literature by using PubMed, EMBASE, and Medline databases. Findings Recommended treatment of BCS is based in an stepwise progressively invasive management strategy that includes anticoagulation and treatment of the underlying prothrombotic risk factors and percutaneous revascularization in short hepatic or inferior vena cava stenosis as first step then, if failure, transjugular intrahepatic portosystemic shunt reserving liver transplantation to those patients that do not respond to previous steps. Conclusions Early diagnosis and treatment have improved the outcome of patients with BCS. However, BCS management remains a challenge, requiring multidisciplinary teams in referral centers with BCS expertise. Keywords Budd-chiari syndrome . Transjugular intrahepatic portosystemic shunt . Anticoagulation . Stepwise management
Abbreviations BCS TIPS LT MPN PV LMWH VKA INR DOACs RCT IVC PTFE
BCS-TIPSS PI Budd-Chiari syndrome Transjugular intrahepatic portosystemic shunt Liver transplant Myeloproliferative neoplasms Policitemia Vera Low molecular weight heparin Vitamin K antagonist International Normalized Ratio Direct oral anticoagulants Randomized clinical trial Inferior vena cava Polytetrafluoroethylene
This article is part of the Topical Collection on Portal Hypertension * Juan Carlos García-Pagán [email protected] 1
Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Villarroel 170, 08036 Barcelona, Spain
2
CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Madrid, Spain
3
The European Reference Network on Rare Liver Disorders (ERN-Liver), Madrid, Spain
Budd-Chiari Syndrome-TIPS Prognosis Index Score
Introduction Budd-Chiari syndrome (BCS) is defined as the obstruction of hepatic venous outflow that may be located from the small hepatic venules up to the entrance of the inferior cava vein into the right atrium. BCS may be secondary to an external compression by a tumor or primary because of thrombosis. Since prognostic and therapeutic implications are very different, we will only focus on primary BCS [1]. Although a rare disorder, diagnosis and adequate management of BCS are relevant because if there is a delay in diagnosis or inadequate treatment, it is associated with high morbi-mortality reducing life expectancy in patients that are usually young or in the middle age [2]. There are no randomized clinica
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