Occlusion of at least two hepatic veins leads to Budd-Chiari syndrome. Single hepatic vein occlusion is usually silent. Obstruction in the venous drainage from liver results in venous congestion causing hepatomegaly. Patients may develop postsinusoidal portal hypertension. Stasis of blood and congestion cause hypoxic damage in the hepatocytes, affecting liver function. This can result in centrilobular fibrosis, nodular regenerative hyperplasia and ultimately cirrhosis. Hepatocellular necrosis results from increased sinusoidal pressure. Budd-Chiari syndrome is commonly associated with atrophy of peripheral regions and enlargement of the caudate lobe because blood is directly shunted through it into the inferior vena cava. Genes associated with increased expression in the pathogenesis of Budd-Chiari syndrome include matrix metalloproteinase 7, superior cervical ganglion 10 (SCG10), proliferating cell nuclear antigen gene, c-myc oncogene, tumor protein p53 gene, thrombospondin-1 gene.
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