The BuddChiari syndrome is a heterogeneous group of disorderscharacterized by hepatic venous outflow obstruction at the levelof the hepatic venules, the large hepatic veins, the inferiorvena cava, or the right atrium.1 Hepatic veno-occlusive diseaserefers to obstruction of hepatic venous outflow at the levelof the central or sublobular hepatic veins, or both.
Pathogenesis
Obstruction of the hepatic venous outflow tract results in increasedhepatic sinusoidal pressure and portal hypertension. In theearly stages, portal venous perfusion of the liver is decreased,which may result in portal venous thrombosis.2 The ensuing venousstasis and congestion lead to hypoxic . . . [Full Text of this Article]
Clinical Manifestations
Diagnosis
Management
Medical Management
Relief of Hepatic Venous Outflow Tract Obstruction
Thrombolytic Therapy and Angioplasty
Transjugular Intrahepatic Portosystemic Shunts
Surgery
Liver Transplantation
Source Information
From the Advanced Liver Disease Study Group, Division of Gastroenterology and Hepatology and Internal Medicine, Mayo Clinic, Rochester, Minn.
Address reprint requests to Dr. Kamath at the Division of Gastroenterology and Hepatology and Internal Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55905, or at kamath.patrick@mayo.edu.
Related Letters:
The BuddChiari Syndrome
Janssen H. L.A., Garcia-Pagan J.-C., Valla D.-C., Cardenas A., Menon K.V. N., Shah V., Kamath P. S.
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N Engl J Med 2004;
350:1906-1908, Apr 29, 2004.
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