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Primary Budd-Chiari syndrome - a single center experience
Joanna Raszeja-Wyszomirska1, Joanna Mieżyńska-Kurtycz, Wojciech Marlicz
1Liver Unit, Pomeranian Medical University, Szczecin, Poland. jorasz@sci.pum.edu.pl
Hepato-Gastroenterology
|July 24, 2012
Summary
Budd-Chiari syndrome (BCS) management requires strict adherence to guidelines. Anticoagulation, TIPS, and liver transplants (OLT) are effective treatments for this rare, potentially lethal condition.
Area of Science:
- Hepatology
- Vascular Medicine
- Thrombosis Research
Background:
- Budd-Chiari syndrome (BCS) is a rare but potentially fatal condition caused by hepatic vein thrombosis.
- Prompt diagnosis and management are critical, as BCS can lead to liver failure within three years if untreated.
Purpose of the Study:
- To retrospectively analyze the diagnostic and therapeutic strategies for Budd-Chiari syndrome.
- To evaluate the outcomes of BCS patients managed between 2004 and 2011.
Main Methods:
- Diagnosis confirmed using contrast CT-angiography and Doppler ultrasound.
- Retrospective analysis of 20 BCS patients managed over an 8-year period.
- Treatment included anticoagulation, Transjugular Intrahepatic Portosystemic Shunt (TIPS), and Orthotopic Liver Transplantation (OLT).
Main Results:
- Myeloproliferative disorders, end-stage liver disease, and protein C deficiency were common causes of BCS.
- The majority of patients received anticoagulation, with some undergoing TIPS or LTx.
- A significant proportion of patients (14/20) were alive and well at least one year post-diagnosis, indicating treatment efficacy.
Conclusions:
- Adherence to diagnostic and therapeutic guidelines is crucial for successful BCS management.
- Anticoagulation, TIPS, and OLT demonstrate efficacy in treating Budd-Chiari syndrome.
- Effective management strategies can significantly improve patient survival and outcomes.
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