Anticoagulation for Budd–Chiari Syndrome

  • Audrey Payancé
  • Aurélie PlessierEmail author


Management of Budd–Chiari syndrome (BCS) is complex, mostly due to the variability of clinical presentation, cumulating bleeding, and thrombotic risk; symptoms vary from asymptomatic to severe portal hypertension and liver failure, in the context of concurring multifactorial prothombotic diseases. These modifications result in precarious hemostatic equilibrium with an increased risk of bleeding and thrombosis in a situation where a multidisciplinary approach combining medical and interventional therapy is considered. For more than 15 years, a stepwise treatment strategy according to response to previous therapy (from less to more invasiveness) has been proposed and is largely used worldwide. The first step consists of pharmacological management with anticoagulation therapy, specific therapy of underlying thrombotic disease, and medical or endoscopic management of liver-related complications. Recanalization of accessible stenosis is systematically considered and performed with angioplasty or stenting when it is feasible. In patients who do not respond to this first step therapy, transjugular intrahepatic portosystemic shunt is proposed, and as a fourth step, orthotopic liver transplantation. This chapter reviews the current rationale, indications, and modalities of anticoagulation therapy in BCS patients considering the balance of efficacy and safety, in this unstable situation of high bleeding vs high recurrent thrombosis risk.


Hepatic vein thrombosis Myeloproliferative neoplasms TIPS Recanalization Portal vein thrombosis JAK2V617F Mutation CYP2C9 VKORC1 Vitamin K antagonists Low molecular weight heparin Severe bleeding 



Budd–Chiari Syndrome


Direct-acting oral anticoagulants


orthotopic liver transplantation


transjugular portosystemic shunt


vitamin K antagonists


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Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Centre de Référence des Maladies Vasculaires du FoieHepatology Unit, Pôle des Maladies de l’Appareil Digestif, Beaujon Hospital, AP-HPClichyFrance
  2. 2.European Reference Network Rare Liver ERN Rare LiverBeaujon Hospital, AP-HPClichyFrance
  3. 3.Filfoie, French Filière de santé Maladies rares du foieBeaujon Hospital, AP-HPClichyFrance
  4. 4.Centre de Recherche sur l’Inflammation CRI, UMR_S1149, Université Paris-Diderot-Paris 7ParisFrance

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