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Inferior vena cava thrombosis at its hepatic portion (obliterative hepatocavopathy)
1Department of Medicine, Chiba University School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, Japan 260-8677. okuda@med.m.chiba-u.ac.jp
Insights
Budd-Chiari syndrome encompasses hepatic vein thrombosis and inferior vena cava (IVC) thrombosis. This study proposes "obliterative hepatocavopathy" as a distinct clinical entity for IVC thrombosis causing liver damage.
Area of Science:
- Vascular Medicine
- Hepatology
- Thrombosis Research
Background:
- Budd-Chiari syndrome traditionally refers to hepatic vein thrombosis but now includes inferior vena cava (IVC) thrombosis.
- Hepatic vein outflow obstruction can result from primary hepatic vein thrombosis or primary IVC thrombosis, considered distinct clinical disorders.
- Primary IVC thrombosis, often in the hepatic portion, can lead to membranous obstruction and congestive liver damage.
Purpose of the Study:
- To differentiate primary hepatic vein thrombosis from primary IVC thrombosis.
- To propose a new term, "obliterative hepatocavopathy," for IVC thrombosis and associated congestive liver damage.
- To describe the distinct clinical and hemodynamic features of obliterative hepatocavopathy compared to hepatic vein thrombosis.
Main Methods:
- Clinical description and comparison of primary hepatic vein thrombosis and primary IVC thrombosis.
- Analysis of hemodynamic differences in hepatic veins, IVC, and portal vein.
- Observation of collateral circulation patterns, particularly the ascending lumbar vein in obliterative hepatocavopathy.
Main Results:
- Primary hepatic vein thrombosis and primary IVC thrombosis are distinct clinical disorders.
- Obliterative hepatocavopathy presents with less severe acute symptoms than hepatic vein thrombosis but involves recurrent thrombosis.
- Distinct hemodynamic profiles and prominent collateral vein dilatation (ascending lumbar vein) characterize obliterative hepatocavopathy.
Conclusions:
- Obliterative hepatocavopathy is proposed as a distinct disease entity separate from hepatic vein thrombosis.
- Understanding these distinctions is crucial for accurate diagnosis and management of hepatic vein outflow obstruction.
- Obliterative hepatocavopathy can lead to congestive liver cirrhosis and potentially hepatocellular carcinoma over time.
Abstract:
The Budd-Chiari syndrome was primarily described as hepatic vein thrombosis within the liver, but it now includes inferior vena cava (IVC) thrombosis and other conditions that cause hepatic vein outflow obstruction. This author and several others maintain that primary hepatic vein thrombosis and primary IVC thrombosis represent two different clinical disorders. Primary thrombosis of the IVC most commonly occurs in its hepatic portion, which seems to be predisposed to thrombosis and has been called membranous obstruction of IVC, because the thrombus organizes into a fibrous and frequently membranous occlusion of the IVC. The hepatic vein orifices are affected to varying degrees, resulting in congestive liver damage. The cause of IVC thrombosis may be a hypercoagulable state such as coagulation factor deficiency and myeloproliferative disorders, but is more often idiopathic. In Nepal, it is endemic with a suspected association with infections. To consider IVC thrombosis and the congestive liver damage as a disease entity, this author proposes the term obliterative hepatocavopathy, separate from hepatic vein thrombosis. Clinically obliterative hepatocavopathy is less severe in its acute phase compared with hepatic vein thrombosis, but it aggravates occlusion of hepatic vein orifices with recurrent thrombosis. Primary hepatic vein thrombosis and obliterative hepatocavopathy display different hemodynamics of the hepatic veins, IVC, and portal vein; dilatation of the subcutaneous veins in the body trunk is more pronounced in obliterative hepatocavopathy because the ascending lumbar vein becomes the major collateral route. Congestive liver cirrhosis develops after a long clinical course that may be complicated by hepatocellular carcinoma.