From the Guidelines
Budd-Chiari syndrome treatment should prioritize anticoagulation with heparin followed by warfarin or direct oral anticoagulants, and may require interventional procedures such as TIPS or liver transplantation in severe cases. The treatment approach should be individualized based on the cause, extent of blockage, and liver damage severity. According to the most recent guidelines 1, patients with Budd-Chiari syndrome should receive anticoagulant therapy as soon as possible for an indefinite period of time to reduce the risk of clot extension and new thrombotic episodes.
Some key considerations in the treatment of Budd-Chiari syndrome include:
- Anticoagulation with low molecular weight heparin (LMWH) for at least 5 to 7 days, and oral anticoagulant treatment with vitamin K antagonists (VKA) aiming at an international normalized ratio (INR) between 2 and 3 1
- Treatment of the underlying prothrombotic cause, such as myeloproliferative disorders, should be initiated concomitantly 1
- Interventional procedures such as angioplasty with stenting or TIPS may be necessary to open blocked hepatic veins or create an alternative blood flow pathway 1
- Liver transplantation may be necessary in severe cases with progressive liver failure 1
It's worth noting that the experience with thrombolysis is limited, and complications can be fatal 1. Additionally, surgical shunts have not demonstrated an independent survival advantage in cohorts of patients with Budd-Chiari syndrome 1.
The most recent study 1 suggests that TIPS creation improves outcomes in patients with Budd-Chiari syndrome who remain symptomatic or without improving liver function despite medical therapy, and is associated with good survival rates. Therefore, TIPS should be strongly considered in these patients.
From the Research
Treatment Options for Budd-Chiari Syndrome
- Medical treatment: Anticoagulation is a key component of treatment, with direct oral anticoagulants (DOACs) being considered effective and safe for long-term anticoagulation in patients with Budd-Chiari syndrome 2, 3.
- Endovascular treatment: Angioplasty, stenting, and local thrombolysis can be used to restore vessel patency 4, 5.
- Transjugular portosystemic shunt (TIPS) placement: TIPS can be used to reduce portal pressure and improve liver function 4, 5.
- Orthotopic liver transplantation: Liver transplantation is considered a last resort treatment for patients with advanced liver disease 4, 5.
Anticoagulation Therapy
- DOACs: Apixaban, rivaroxaban, and dabigatran have been used in patients with Budd-Chiari syndrome, with reported efficacy and safety 2, 3.
- Vitamin K antagonists (VKAs): VKAs, such as warfarin, have been traditionally used for anticoagulation in patients with Budd-Chiari syndrome, but may have a higher risk of bleeding compared to DOACs 4, 6.
- Low-molecular-weight heparin (LMWH): LMWH can be used as a bridge to oral anticoagulation or in patients who are not candidates for DOACs or VKAs 4, 6.
Complications and Risks
- Bleeding: Anticoagulation therapy can increase the risk of bleeding, with major bleeding events reported in patients treated with DOACs and VKAs 2, 3.
- Thrombosis: Patients with Budd-Chiari syndrome are at risk of thrombosis, particularly in the hepatic veins and inferior vena cava 4, 5.
- Liver disease: Budd-Chiari syndrome can lead to advanced liver disease, including cirrhosis and liver failure, if left untreated or inadequately treated 4, 5.