From the Guidelines
Budd-Chiari thrombosis is a rare condition characterized by the obstruction of the hepatic veins or the inferior vena cava, which requires immediate anticoagulation therapy to prevent further thrombosis and allow for potential recanalization of occluded vessels. According to the EASL clinical practice guidelines 1, patients with Budd-Chiari syndrome (BCS) should receive anticoagulant therapy as soon as possible for an indefinite period of time. The recommended treatment includes low molecular weight heparin (LMWH) for at least 5 to 7 days, followed by oral anticoagulant treatment with vitamin K antagonists (VKA), aiming at an international normalized ratio (INR) between 2 and 3.
Key aspects of management include:
- Treating underlying prothrombotic causes, such as myeloproliferative disorders, concomitantly with anticoagulation therapy 1
- Considering thrombolysis for acute cases with severe symptoms, although the experience with this approach is limited and complications can be fatal 1
- Managing complications like ascites and portal hypertension, which are common in patients with BCS
- Evaluating the potential need for transjugular intrahepatic portosystemic shunt (TIPS) or liver transplantation in severe cases, as these interventions can improve survival and quality of life 1
It is essential to note that the treatment of BCS should be individualized, and the decision to use TIPS or liver transplantation should be based on the patient's specific clinical characteristics and response to medical therapy. As stated in the guidelines 1, TIPS has a lower morbidity and mortality rate than surgery and is feasible in most patients with IVC obstruction and in those with severe IVC stenosis. However, the placement of previous TIPS can make a posterior liver transplantation more difficult if it is needed, although this has not been confirmed in more recent studies 1.
From the Research
Definition of Budd-Chiari Thrombosis
Budd-Chiari syndrome (BCS) is a clinical manifestation of various prothrombotic conditions, resulting from thrombosis of the hepatic veins 2. It is a rare and life-threatening disorder that can be lethal within 3 years of the onset of symptoms if untreated 2.
Causes of Budd-Chiari Thrombosis
The causes of BCS include:
- Myeloproliferative disorders 2, 3
- End-stage liver disease 2
- Protein C deficiency 2
- Paroxysmal nocturnal hemoglobinuria (PNH) 2
- Antiphospholipid syndrome 2
- Secondary poliglobulia 2
Treatment of Budd-Chiari Thrombosis
The treatment of BCS includes:
- Anticoagulation therapy with warfarin or low-molecular-weight heparin (LMWH) 2, 3, 4
- Radiological interventions such as transjugular intrahepatic portosystemic shunt (TIPS) and/or angioplasty/stenting 3
- Liver transplantation (LTx) 2
- Direct oral anticoagulants (DOACs) may also be effective in the anticoagulation of patients with BCS 5
Management and Outcomes
The management of BCS requires strict adherence to diagnostic and therapeutic guidelines 2. Early radiological intervention and hematological screening are associated with excellent outcomes in BCS 3. The overall survival rate for BCS patients can be as high as 96% at 1 year and 81% at 5 years with appropriate treatment 3.