Hemodynamic Liver Diseases

2021-02-22 12:00 AM

Budd-Chiari syndrome (hepatic vein thrombosis) refers to occlusion of the hepatic vein by a thrombus, often resulting in death.

HEMODYNAMIC LIVER DISEASES

Budd-Chiari syndrome (hepatic vein thrombosis) refers to occlusion of the hepatic vein by a thrombus, often resulting in death. While a few cases are idiopathic, more often there is an underlying process predisposing for the thrombosis e.g., polycythemia vera, pregnancy, oral contraceptives, paroxysmal nocturnal hemoglobinuria, or hepatocellular carcinoma. It presents with abdominal pain, hepatomegaly, ascites, jaundice, splenomegaly, and in some cases, death.

The initial diagnostic test is ultrasonography. Microscopically, the liver shows centrilobular congestion and necrosis. In the chronic form, fibrosis develops. Treatment includes supportive care and treatment of the underlying condition. Some patients require lifelong anticoagulation.

Chronic passive congestion of the liver refers to a “backup of blood” into the liver, usually due to right-sided heart failure. Grossly, the liver characteristically has a nutmeg pattern of alternating dark (congested central areas) and light (portal tract areas) liver parenchyma. Microscopically, the liver shows centrilobular congestion.

Complications include centrilobular necrosis, which is the ischemic necrosis of centrilobular hepatocytes. Long-standing congestion can lead to centrilobular fibrosis, which can eventually become cardiac cirrhosis (sclerosis).