Learn About Budd-Chiari Syndrome

What is the definition of Budd-Chiari Syndrome?
Budd-Chiari syndrome is a rare disorder characterized by obstruction of the veins of the liver that carry the blood flow from the liver. When the blood flow out of the liver is impeded, blood backs up in the liver, causing it to enlarge (hepatomegaly). The spleen may also enlarge (splenomegaly). This backup of blood increases blood pressure in the portal vein, which carries blood to the liver from the intestines (portal hypertension), and result in  dilated, twisted veins in the esophagus (esophageal varices). Portal hypertension, leads to fluid accumulating in the abdomen (called ascites). The clot may extend to also block the inferior vena cava (the large vein that carries blood from the lower parts of the body to the heart). Varicose veins in the abdomen near the skin’s surface may develop and become visible. In some cases,  scarring of the liver (cirrhosis) occurs. Other symptoms may include fatigue, abdominal pain, nausea, jaundice and bleeding in the esophagus. The severity of the disorder varies from case to case, depending on the site and number of affected veins. It most often occurs in patients which have a disorder that makes blood more likely to clot, such as those who are pregnant or who have a tumor, a chronic inflammatory disease, a clotting disorder, an infection, or a myeloproliferative disorder. In about one third of the cases, the cause of Budd-Chiari syndrome is unknown. Drugs or surgical interventions may be used to dissolve or decrease the size of the obstruction (if it is a clot). In some cases liver transplantation is needed.  Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD).
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What are the alternative names for Budd-Chiari Syndrome?
  • Membranous obstruction of the inferior vena cava
What are the current treatments for Budd-Chiari Syndrome?
The treatment of Budd-Chiari syndrome varies, depending on the cause of the blockage. Medical treatments may include:
  • Blood-thinning (anticoagulation) medications
  • Clot-busting drugs (thrombolytic treatment)
  • Treatment for the liver disease, including ascites

Surgical treatments may also be considered and include:

  • Angioplasty and stent placement
  • Transjugular intrahepatic portosystemic shunt (TIPS)
  • Venous shunt surgery

While medical therapy can be instituted for short-term, symptomatic benefit, medical therapy alone has been associated with a high 2-year mortality rate (80-85%).

In cases of severe cirrhosis liver transplant may be needed. You can view more detailed information regarding the medical and surgical options for treatment of Budd-Chiari syndrome by visiting the Medscape Reference website on Budd-Chiari syndrome treatment & managment. You may need to register to view the article, but registration is free.

Who are the top Budd-Chiari Syndrome Local Doctors?
SG
Shivanand R. Gamanagatti
Elite
Highly rated in
10
conditions

All India Institute Of Medical Sciences

New Delhi, DL, IN 

Shivanand Gamanagatti is in New Delhi, India. Gamanagatti is rated as an Elite expert by MediFind in the treatment of Budd-Chiari Syndrome. They are also highly rated in 10 other conditions, according to our data. Their top areas of expertise are Budd-Chiari Syndrome, Mesenteric Venous Thrombosis, Liver Cancer, and Chondroblastoma.

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XQ
Xingshun K. Qi
Elite
Highly rated in
20
conditions

General Hospital Of Shenyang Military Area

Shenyang, CN 

Xingshun Qi is in Shenyang, China. Qi is rated as an Elite expert by MediFind in the treatment of Budd-Chiari Syndrome. They are also highly rated in 20 other conditions, according to our data. Their top areas of expertise are Budd-Chiari Syndrome, Cirrhosis, Mesenteric Venous Thrombosis, and Portal Hypertension.

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AM
Andrea P. Mancuso
Elite
Highly rated in
13
conditions

Medicina Interna 1

Palermo, IT 

Andrea Mancuso is in Palermo, Italy. Mancuso is rated as an Elite expert by MediFind in the treatment of Budd-Chiari Syndrome. She is also highly rated in 13 other conditions, according to our data. Her top areas of expertise are Budd-Chiari Syndrome, Mesenteric Venous Thrombosis, Cirrhosis, and Liver Transplant.

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What are the latest Budd-Chiari Syndrome Clinical Trials?
The Perioperative Liberal or Restrictive Transfusion in Pediatric Liver Transplantation: A Prospective Randomized Controlled Trial
Status: Recruiting
Start Date: December 7, 2016
Intervention Type: Other
Phase: Not Applicable
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Summary: The patients assigned to the restrictive group will receive transfusion once the hemoglobin concentration falls below 6.0 g per deciliter, with a target hemoglobin range of 7.5 to 8.0 g per deciliter. Patients assigned to the liberal group will receive transfusion once the hemoglobin concentration falls below 8.0 g per deciliter, with a target hemoglobin range of 9.0 to 10.0 g per deciliter.~The p...
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Prospective Evaluation of Coagulation Status and Thromboelastometry Guided Management of Genetic and Acquired Thrombophilia in Patients With Portal Vein Thrombosis and Budd-Chiari Syndrome
Status: Recruiting
Start Date: October 15, 2021
Intervention Type: Diagnostic Test
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Summary: Portal vein thrombosis is defined as partial or complete occlusion of the portal vein lumen by the blood clot or its replacement by multiple collateral vessels with the hepato-petal flow, known as 'portal cavernoma'. [1,2] Based on the published literature, 15-25% of patients with cirrhosis have portal vein thrombosis (PVT) [3], and 35-50% of patients with hepatocellular carcinoma (HCC) have malig...
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What are the Latest Advances for Budd-Chiari Syndrome?
Analysis of curative efficacy of transjugular intrahepatic portosystemic shunt using viabahn stent in the treatment of Budd-Chiari syndrome with extensive hepatic vein occlusion.
Condition: Budd-Chiari Syndrome (BCS) with Extensive Hepatic Vein Occlusion
Journal: Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
Treatment Used: Transjugular Intrahepatic Portosystemic Shunt (TIPS) Using Viabahn Stent
Number of Patients: 25
Published: November 24, 2021
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Summary: This study investigated the effectiveness of transjugular intrahepatic portosystemic shunt (TIPS) using viabahn stent in patients with Budd-Chiari syndrome (BCS) with extensive hepatic vein occlusion.
Effect of thrombolytic therapy and influencing factors of Budd-Chiari syndrome with inferior vena cava thrombosis.
Condition: Budd-Chiari Syndrome (B-CS) with Inferior Vena Cava (IVC) Thrombosis
Journal: Zhonghua wai ke za zhi [Chinese journal of surgery]
Treatment Used: Thrombolytic Therapy
Number of Patients: 67
Published: November 07, 2021
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Summary: This study examined the effect of thrombolytic therapy (the use of drugs to break up or dissolve blood clots) for Budd-Chiari syndrome (veins that drain the liver are blocked or narrowed by a clot; B-CS) with inferior vena cava (IVC) thrombosis (blood clot impedes the blood flow through the inferior vena cava, a large vein in the body).
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Phase II Study of Concurrent Sorafenib and Radiotherapy for Advanced Hepatocellular Carcinoma With Portal Vein and/or Hepatic Vein Tumor Thrombosis.
Condition: Advanced Hepatocellular Carcinoma (HCC) with Portal Vein and/or Hepatic Vein Tumor Thrombosis
Journal: International journal of radiation oncology, biology, physics
Treatment Used: Concurrent Sorafenib and Radiotherapy
Number of Patients: 86
Published: October 27, 2021
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Summary: This study investigated the safety and effectiveness of concurrent sorafenib and radiotherapy for the treatment of patients with advanced hepatocellular carcinoma (liver cancer; HCC) with portal vein and/or hepatic vein tumor thrombosis.
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