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Budd Chiari Syndrome

About Budd Chiari Syndrome

(Chiari's Disease, Hepatic Vein Thrombosis, HVT)

Budd Chiari syndrome is a rare disorder that causes hepatic vein obstruction. The hepatic vein is responsible for carrying blood away from the liver and back to the heart.

Why it is Problematic

Hepatic vein obstruction caused by a tumor or blood clot prevents blood from flowing out of the liver and back to the heart. There is the potential for liver damage when the liver does not receive adequate oxygen. Budd Chiari syndrome can be life threatening. Individuals with liver failure may only live up to 3 years without a liver transplant. Life expectancy depends on the blockage location, the speed with which treatment is received, the type of treatment, and the underlying cause of Budd Chiari syndrome.

Causes and Risk Factors

Budd Chiari syndrome is often caused by conditions that increase the likelihood of blood clot formation such as:

Abnormal cell growth in bone marrow (myeloproliferative disorders)
Cancer
Autoimmune disease
Chronic inflammatory disease
Infection
Oral contraceptives
Pregnancy
Cigarette smoking
Blood disorders
Liver trauma
Vein inflammation (phlebitis)
Immunosuppressive drugs

Symptoms

Budd Chiari syndrome can be symptomless in some individuals. When symptoms are present, they may include:

Abdominal swelling or stretching
Pain in the right upper abdomen
Vomiting blood
Jaundice (yellowing of the skin and whites of the eyes)
Ascites
Nausea
Unexplained weight loss
Enlarged spleen

Diagnosis

A blood test can determine how well the liver is functioning. Detection of liver damage may require diagnostic imaging tests such as a CT scan, MRI, or ultrasound. A procedure called hepatic vein catheterization can identify pressure within the heaptic vein. In this procedure, a small instrument is inserted through a catheter into the hepatic vein. Additionally, a liver biopsy may be used to diagnose Budd Chiari syndrome.

Treatment and Prevention

Treatment varies depending on the cause of the blockage. Your doctor may recommend blood thinning medication (anticoagulants), medicine to break up blood clots (thrombolytic treatment), or medication for specific symptoms of liver disease, such as ascites.

Surgery may be recommended to unblock the veins and may include angioplasty (a balloon catheter used to open up the blocked hepatic vein), and stent placement (to reroute blood flow around the clotted vein and into the larger vein called the vena cava), liver transplantation (for those patients who have deteriorating liver function and complications such as cirrhosis), transjugular intrahepatic portosystemic shunt (TIPS) and venous shunt surgery.

Seek immediate medical attention if you experience any of the symptoms of Budd Chiari syndrome or if you are being treated for Budd Chiari syndrome and develop new symptoms.

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