In the Budd Chiari syndrome hepatic venous outflow typically presents with ascites, sometimes accompanied by pain over the liver. Interventional radiology produces excellent results from treatment by hepatic vein angioplasty and transjugular intrahepatic portosystemic shunt (TIPS). Haematological investigations have a high yield revealing a prothrombotic condition such as Factor V Leidenn (circa 25%) and myeloproliferative disease. Hepatic venoocclusive disease is typically due to an inflammatory and fibrotic reaction which occludes the hepatic sinusoids. Historically, it was associated with a variety of toxic injuries (e.g. bush tea disease), but now almost exclusively in the context of bone marrow transplantation. Typically portal vein thrombosis presented with bleeding varices, but increasingly it is recognized as a cause of cholestatic jaundice with choledocholithiasis from portal biliopathy. The hepatopulmonary syndrome is characterized by dilatation of pulmonary capillaries to such an extent that oxygen is unable to diffuse adequately to prevent hypoxemia. The diagnosis of portopulmonary hypertension requires a demonstration of increased pulmonary vascular resistance. Congential and hereditary vascular anomalies include persistent patency of the ductus venosus. Liver involvement by hereditary haemorrhagic telangiectasia may produce symptoms from high output cardiac failure, portal hypertension or biliary disease. Both benign and malignant primary vascular lesions also occur.
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