The Budd–Chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the level of the hepatic venules, the large hepatic veins, the inferior vena cava, or the right atrium.1 Hepatic veno-occlusive disease refers to obstruction of hepatic venous outflow at the level of the central or sublobular hepatic veins, or both.
Pathogenesis
Obstruction of the hepatic venous outflow tract results in increased hepatic sinusoidal pressure and portal hypertension. In the early stages, portal venous perfusion of the liver is decreased, which may result in portal venous thrombosis.2 The ensuing venous stasis and congestion lead to hypoxic . . .
http://content.nejm.org/cgi/content/full/350/6/578
The Budd–Chiari Syndrome
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