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Budd-Chiari Syndrome: Update on Classification and Intravascular US
Anne Sailer1, Nariman Nezami2, Parham Tabeli Bouroujeni3
1Section of Interventional Radiology, Department of Radiology and Biomedical Imaging, Yale School of Medicine, 333 Cedar St, PO Box 208042, Room TE-2, New Haven, CT 06520.
Insights
Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction. Understanding the BCS classification system and using intravascular ultrasound (IVUS) aids in guiding effective interventional therapies for BCS.
Area of Science:
- Interventional Radiology
- Hepatology
- Vascular Medicine
Background:
- Budd-Chiari syndrome (BCS) arises from hepatic venous outflow obstruction, leading to sinusoidal congestion, portal hypertension, and potential cirrhosis.
- Obstruction can affect hepatic veins (HVs) or the inferior vena cava (IVC), presenting globally or segmentally.
- Effective treatment necessitates a thorough understanding of causal factors, lesion characteristics, clinical status, and liver function.
Purpose of the Study:
- To highlight the importance of the Budd-Chiari syndrome (BCS) classification system in guiding interventional therapy.
- To underscore the utility of intravascular ultrasound (IVUS) in characterizing BCS lesions and aiding treatment decisions.
- To emphasize the role of radiologists in understanding the BCS classification for optimal patient management.
Main Methods:
- Review and application of the established Budd-Chiari syndrome (BCS) classification system.
- Utilization of intravascular ultrasound (IVUS) for detailed lesion characterization and procedural guidance.
- Analysis of treatment strategies including angioplasty, stent placement, and portosystemic shunt creation.
Main Results:
- The BCS classification system provides a framework for standardizing diagnosis and interventional therapy.
- IVUS aids in BCS lesion characterization, guiding decisions on stent placement and portosystemic shunt creation.
- IVUS can improve procedural efficiency and success rates while reducing radiation and contrast agent use.
Conclusions:
- A comprehensive understanding of the BCS classification system is essential for radiologists.
- Intravascular ultrasound (IVUS) is a valuable tool for characterizing BCS lesions and optimizing interventional procedures.
- Integrating the BCS classification with IVUS facilitates tailored and effective treatment strategies for Budd-Chiari syndrome.
Abstract:
Budd-Chiari syndrome (BCS) results from the obstruction of hepatic venous outflow and can occur anywhere from the level of the small hepatic veins (HVs) to the junction of the inferior vena cava (IVC) and the right atrium. The obstruction of the HVs can be global or segmental. This results in sinusoidal congestion, which leads to portal hypertension and subsequent centrilobular fibrosis, nodular regenerative hyperplasia, and ultimately cirrhosis. Treatment is aimed at restoring HV or IVC outflow by angioplasty, stent placement, or creating a portosystemic shunt, which in turn drastically reduces hepatic sinusoidal pressure. Thus, treatment strategies should be based on detailed knowledge of the underlying causal factors, lesion classification, clinical presentation, and baseline liver function. The BCS classification system is divided into types and subtypes that can guide interventional therapy, with the aim of standardizing the imaging diagnosis and interventional therapy of BCS. Intravascular US (IVUS) aids in BCS lesion characterization as described by the BCS classification system. It can help determine the need for stent placement following angioplasty and is helpful in portosystemic shunt creation. Additionally, IVUS can reduce radiation dose, contrast agent volume, and procedure time and increase the technical success rate of procedures. It is essential for the radiologist to understand the BCS classification system as a useful tool to guide interventional therapy. ©RSNA, 2025 Supplemental material is available for this article.
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