Budd-Chiari syndrome

Includes hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava and the right atrium, regardless of the cause of the obstruction.

Classic triad of symptoms is abdominal pain, ascites, and hepatomegaly.

Seventy-five percent of patients have at least one underlying hypercoagulable state.

Color and pulsed Doppler ultrasonography is the first-line investigation for diagnosis. Patients should also undergo testing for hypercoagulable states to identify any predisposition to venous thrombosis.

The aim of therapy and interventional management is to relieve hepatic congestion and prevent necrosis, fibrosis, and ultimately liver failure, cirrhosis, and/or portal hypertension.

The main treatment options are radiologic interventional procedures including angioplasty of hepatic vein and/or inferior vena cava, or transjugular intrahepatic portosystemic shunts (TIPS). Further to this, surgical procedures may be used, such as surgical shunts and liver transplantation.

Medical treatment in the form of anticoagulation therapy, diuretics, and treatment of underlying thrombophilic condition is given parallel to interventional therapy.

Untreated, the syndrome has a reported mortality of 80%. In the past few decades, prognosis has dramatically improved due to advances and new modalities in diagnosis and treatment of the disease.

International expert panels have agreed that “Budd-Chiari syndrome” should be used as an eponym for “hepatic venous outflow tract obstruction”, independent of the level or mechanism of obstruction.[1]DeLeve LD, Valla DC, Garcia-Tsao G; American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology. 2009;49:1729-1764. http://onlinelibrary.wiley.com/doi/10.1002/hep.22772/full http://www.ncbi.nlm.nih.gov/pubmed/19399912?tool=bestpractice.com Cardiac and pericardial diseases, and sinusoidal obstruction syndrome are excluded from this definition.