What is the pathophysiology of Budd-Chiari syndrome?

Updated: Oct 10, 2018
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD  more...
  • Print

Occlusion of a single hepatic vein is usually silent. Overt Budd-Chiari syndrome generally requires the occlusion of at least 2 hepatic veins. [8] Venous congestion of the liver causes hepatomegaly, which can stretch the liver capsule and be very painful. Enlargement of the caudate lobe is common because blood is shunted through it directly into the inferior vena cava (IVC).

Hepatic function can be affected to a degree, depending on the amount of stasis and the resultant hypoxia. Increased sinusoidal pressure can itself cause hepatocellular necrosis. [49] The literature also suggests that upregulation of specific genes in chronic Budd-Chiari syndrome contributes to liver destruction through the stimulation of extracellular matrix proliferation, which contributes to liver fibrosis.

The most prominent genes involved include matrix metalloproteinase 7 and superior cervical ganglion 10 (SCG10), which are increased in expression, and thrombospondin-1, which is decreased. [9] Overexpression of the proliferating cell nuclear antigen gene, the c -MYC oncogene, and the tumor protein p53 gene may also be etiologic factors for Budd-Chiari syndrome. [10]

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!