Budd-Chiari Syndrome Overview


An uncommon condition, Budd-Chiari syndrome (BCS) syndrome is caused by blood clots that narrow completely or partially block the large veins that carry blood from the liver to the heart.

Budd-Chiari occurs in people of all races and while it’s somewhat more common in women, both sexes can become afflicted.

Age of onset for the disease is usually in the 30s-40s, but it does occur in people of all ages.

Budd-Chiari Syndrome Signs and Symptoms

Symptoms of Budd-Chiari vary depending on whether they appear suddenly, or develop more gradually. Most often, symptoms develop slowly over a period of weeks or months.

Generally, BCS is characterized by liver enlargement (hepatomegaly), an abnormal collection of fluid within the abdomen (ascites), and abdominal pain.

These symptoms are a result of impeded blood flow from the liver, causing blood to accumulate in the liver and enlarging it.

The spleen may also become enlarged, and sometimes severe bleeding occurs in the esophagus resulting in vomiting of blood or blood in the stool.

Other Symptoms:

  • Ankle / leg swelling
  • Coma
  • Confusion
  • Elevated liver enzymes
  • Fatigue
  • Hepatic encephalopathy: behavioral, neurological, and psychological changes associated with advanced liver disease
  • Jaundice
  • Lactic acidosis (decreasing the blood pH level below normal)
  • Liver failure
  • Nausea and vomiting
  • Prominence of collateral veins (appearance of spider veins on the abdominal wall or prominence/swelling of the belly button)

Some people, however, develop no symptoms whatsoever.

Budd-Chiari Syndrome Causes and Risk Factors

Usually, the cause of Budd-Chiari syndrome is a condition that makes it more likely for blood to clot.

Hematological disorders:

  • Clotting disorders
  • Excess red cells (polycythemia vera)
  • Premature breakdown of red blood cells (paroxysmal nocturnal hemoglobinuria)
  • Sickle cell disease

Chronic infections:

  • Amebic abscess
  • Aspergillosis
  • Hydatid cysts
  • Syphilis
  • Tuberculosis

Chronic inflammatory diseases:

  • Behçet disease
  • Inflammatory bowel disease
  • Mixed connective-tissue disease
  • Sarcoidosis
  • Sjögren syndrome
  • Systemic lupus erythematosus

Inherited thrombotic diathesis:

  • Antithrombin III deficiency
  • Factor V Leiden deficiency
  • Protein C deficiency
  • Protein S deficiency

Tumors:

  • Adrenal carcinoma
  • Hepatocellular carcinoma (liver cancer)
  • Leiomyosarcoma (malignant smooth muscle tumor)
  • Renal cell carcinoma (kidney cancer)
  • Right atrial myxoma (non-cancerous heart tumor)
  • Wilms tumor (kidney cancer that occurs more commonly in children)

Other Causes:

  • Alpha1-antitrypsin deficiency
  • Dacarbazine (chemotherapy drug)
  • Oral contraceptives
  • Parasitic infections
  • Pregnancy and postpartum
  • Trauma and injury

Budd-Chiari syndrome can begin suddenly and rather severely during pregnancy because at this time a woman’s blood clots more readily.

In approximately one third of BCS patients, the underlying cause is unclear.

Budd-Chiari Syndrome Complications

The congestion of blood in the veins carrying blood away from the liver causes blood pressure in the portal vein (a large vein that carries blood to the liver from the intestines) to increase.

Known as portal hypertension, this increased pressure can result in varicose veins in the esophagus as well as in the abdomen near the skin’s surface. If BCS is left untreated, bleeding from varicose veins in the esophagus can result, and this can be life threatening.

Eventually, severe scarring of the liver (cirrhosis) also occurs.

Diagnosing Budd-Chiari Syndrome

BCS may be suspected when there is an abnormal amount of fluid in the abdomen, liver enlargement and/or failure, or cirrhosis when testing shows no obvious cause.

Abnormal results of liver function tests may also indicate BCS.

Liver enzyme levels and other organ markers (creatinine, electrolytes, lactic acid dehydrogenase/LDH, and urea) are measured when BCS is suspected.

A liver biopsy may be done to rule out other etiologies of cirrhosis like fatty liver disease or an infiltrative disorder and to determine if cirrhosis has developed.

Further Testing for BCS can include: 

  • Doppler ultrasonography
  • Magnetic resonance imaging of blood vessels (magnetic resonance angiography)
  • Computed tomography (CT scan)

These tests are able to evaluate the blood flow in the portal vein (that carries blood to the liver) and the hepatic veins that carry blood from the liver to the heart (and where the blood clots in Budd-Chiari).

When surgery is indicated, venography is necessary. X-rays of the veins are taken after a radiopaque dye is injected into a vein in the groin.

Budd-Chiari Treatment and Prognosis

Treatment depends on how severe the BCS is, and how quickly the illness has developed. A small percentage of patients can be treated through symptom management: sodium restriction and diuretics to control excessive abdominal fluid build-up, and anticoagulants such as heparin and warfarin to prevent the formation of blood clots.

Most people, however, require more complex medical intervention.

Surgical shunts – used to divert blood flow around the obstruction – are an option for milder forms of BCS. For best results, shunts must be placed relatively soon after diagnosis.

Shunts sometimes become blocked, especially in those prone to forming blood clots. Shunts also increase the risk of cognitive issues related to liver dysfunction due to the blood bypassing the liver, so toxins are not as readily removed.

Another option is the transjugular intrahepatic portosystemic shunt (TIPS). Similar to a surgical shunt, this intervention has a lower procedure-related mortality rate.

Life-saving liver transplantation may be necessary for patients with severe liver failure.

Most people need to take anticoagulants indefinitely to prevent new blockages from developing, enlarging, or recurring.

If left untreated, Budd-Chiari syndrome is potentially fatal. While life expectancy varies with partial vein blockages, if a completely blocked vein is left untreated, most people die of liver failure within three years.

The underlying cause of BCS affects survival rate.  Generally, close to two thirds of BCS patients survive for ten years.

Learn more about other liver-related diseases and conditions:

Julie C. Guider MyGoodGut

Medically reviewed by Julie Guider, M.D.

Dr. Julie Guider earned her medical degree from Louisiana State University School of Medicine. She completed residency in internal medicine at the University of Virginia. She completed her general gastroenterology and advanced endoscopy fellowships at University of Texas-Houston. She is a member of several national GI societies including the AGA, ACG, and ASGE as well as state and local medical societies.