Budd-Chiari Syndrome

What is Budd-Chiari syndrome?

Budd-Chiari syndrome involves narrowing or a blockage (obstruction) in one or more of the veins that drain your liver (hepatic veins). The narrowing can be from inflammation within your veins or pressure (compression) outside your veins. An obstruction may block all of a vein or only part of it. The obstruction can be from a blood clot, but not always.

Budd-Chiari can cause blood to back up into your liver, enlarging it. Your spleen may also enlarge.

Budd-Chiari syndrome may also cause other conditions, including:

• Portal hypertension: Increased pressure in your portal vein. This vein carries blood from your digestive tract and spleen to your liver.
• Varices: Enlarged veins in your esophagus, stomach or rectum.
• Ascites: A buildup of fluid in your abdomen.
• Cirrhosis: Irreversible scarring of your liver.

Types of Budd-Chiari syndrome

Providers classify Budd-Chiari syndrome based on how long you’ve had it and the severity of your symptoms. Types include:

• Acute Budd-Chiari syndrome with acute liver failure: Symptoms develop quickly (within days or weeks), and your liver function rapidly declines.
• Acute Budd-Chiari syndrome without liver failure: Symptoms develop quickly but without loss of liver function.
• Subacute Budd-Chiari syndrome: Symptoms set in more slowly, usually over several months. This is the most common type of Budd-Chiari syndrome.
• Chronic Budd-Chiari syndrome: Symptoms usually aren’t noticeable until the blockage has already caused irreversible scarring (cirrhosis) in your liver.

Budd-Chiari syndrome may be primary or secondary:

• Primary Budd-Chiari syndrome occurs because of a narrowing or blockage within your blood vessels. Causes include a blood clot (bland thrombus) or a blockage from a collection of tumor cells. Medical conditions that cause inflammation of your hepatic veins may also contribute.
• Secondary Budd-Chiari syndrome occurs because of an issue outside of your blood vessels. Causes include a malignant (cancerous) or benign (noncancerous) tumor placing pressure on one of your hepatic veins or inferior vena cava (IVC). Your IVC drains blood from your hepatic veins into the right side of your heart. Conditions that injure liver cells or place pressure on hepatic veins can also be a cause. These include certain fungal infections, trauma or chemotherapy.

What are the symptoms of Budd-Chiari syndrome?

The signs and symptoms of Budd-Chiari syndrome include:

• Abdominal pain (particularly in your right upper abdomen).
• Ascites (swelling in your abdomen caused by excess fluid).
• Jaundice (your skin, whites of your eyes and tongue turn yellow).
• Hepatic encephalopathy (confusion or coma caused by liver disease).
• Hepatomegaly (enlarged liver).
• Splenomegaly (enlarged spleen).
• Bleeding from your esophagus, stomach or rectum.
• Edema (swelling) in your legs.
• Fatigue (extreme tiredness).
• Liver failure (liver no longer functions adequately).

The timing of symptoms depends on the type of Budd-Chiari syndrome you have. People with acute forms typically notice symptoms more quickly than people with subacute or chronic Budd-Chiari syndrome. Researchers estimate that 15% to 20% of people with subacute and chronic forms don’t have symptoms.

What causes Budd-Chiari syndrome?

Conditions and situations that lead to blood clots cause Budd-Chiari syndrome. These include:

• Myeloproliferative neoplasms (MPNs): Rare blood cancers that cause your body to make too many blood cells. MPNs include polycythemia vera, myelofibrosis and essential thrombocytosis.
• Hypercoagulable disorders: Conditions that cause excess clot formation. These include protein C or S deficiency, antiphospholipid antibody syndrome, antithrombin III deficiency, Factor V Leiden mutation and lupus.
• Masses in your liver or kidneys: Malignant or benign masses that can cause compression of your hepatic veins or IVC.
• Sickle cell disease: A blood disease in which red blood cells change shape from round to sickle-shaped.
• Inflammatory bowel disease (IBD): A group of disorders that cause inflammation in your digestive system.
• Pregnancy: Pregnancy increases your risk of blood clots. This is especially the case if you’re pregnant and also have a condition that predisposes you to blood clots.
• Oral contraceptives (birth control pills): Some forms of birth control increase your risk of blood clots.

One in five cases of Budd-Chiari syndrome is idiopathic. This means healthcare providers can’t determine a clear cause for the condition.

How is Budd-Chiari syndrome diagnosed?

A healthcare provider can diagnose Budd-Chiari syndrome through a physical exam and tests. They’ll ask about your symptoms and look for signs of Budd-Chiari syndrome, like upper abdominal pain and swelling. They’ll arrange for blood tests to check your liver function and see if you’re at risk for blood clots. Up to 50% of people with Budd-Chiari syndrome have high liver enzyme levels.

Imaging tests can show if there’s narrowing or obstruction of your hepatic veins or IVC. These tests include:

• Doppler ultrasound: This test uses sound waves to record images of your liver, spleen and kidneys. It can show problems in your blood vessels that may signal Budd-Chiari syndrome, like irregular blood flow in your veins. It’s often the first and only test needed to diagnose Budd-Chiari syndrome.
• Computed tomography (CT) scan: This test uses X-rays and computers to produce images of a cross-section of your body. It can show signs of Budd-Chiari syndrome, including an enlarged liver and ascites. For this procedure, you may need an injection of a contrast material. This is a safe substance that makes abnormalities in your blood vessels show up more clearly in the pictures. Your provider may check your kidney function tests before ordering this type of imaging.
• Magnetic resonance imaging (MRI): This test uses a large magnet, radio waves and a computer to produce very clear pictures of the inside of your body. This testing may also require the use of contrast material.
• Venography (venogram): This test uses X-rays to show blood flow in your veins. You may need venography if the other tests haven’t confirmed your diagnosis, but your healthcare provider believes you likely have Budd-Chiari syndrome. Unlike a Doppler ultrasound (which also shows blood flow), a venogram is invasive. A provider will need to inject a contrast material (a safe dye) into your veins so they can see blood flow on the X-ray.

Your provider (a radiologist or hepatologist) may order a liver biopsy to see if you have cirrhosis. A sample of your liver tissue will be sent to the pathology lab so a pathologist can examine it under a microscope.

How is Budd-Chiari syndrome treated?

Treatments for Budd-Chiari syndrome dissolve clots or improve narrowing in your hepatic veins or IVC to decrease swelling and scar formation in your liver. Whenever possible, treatments involve managing the underlying condition that caused you to develop clots. Also, managing complications, like portal hypertension (high blood pressure in your portal vein), is essential.

Medications

Your healthcare provider may prescribe blood thinners (anticoagulants), such as heparin or warfarin, to dissolve the blood clots.

Nonsurgical procedures

You may need a procedure to help redirect blood flow and relieve pressure in your blood vessels. Procedures include:

• Transjugular intrahepatic portosystemic shunt (TIPS): A healthcare provider makes a tunnel through your liver with a needle. Then, they connect the portal vein (the vein that carries blood to your liver) to one of your hepatic veins (the three veins that carry blood from your liver). They’ll use X-ray imaging to guide them as they place a stent (tiny metal tube) into this tunnel to keep the track open.
• Percutaneous transluminal angioplasty: A healthcare provider inserts a catheter (a thin, hollow tube with a balloon at the tip) through your skin and into a blood vessel. They’ll guide the catheter to the clot or narrowed area. When the catheter reaches the affected area, your provider will inflate the balloon to widen the vein. They may place a stent at the site to keep the vein open.

Liver transplant

You may need a liver transplant if other treatments haven’t helped or aren’t an option. For example, you’ll likely need a liver transplant if you have liver failure, cirrhosis of the liver, bleeding from varices or ascites that can’t be treated with medications.

What is the prognosis (outlook) for people with Budd-Chiari syndrome?

Your prognosis depends on various factors, including how severe your condition is and your response to treatment. The outlook tends to be better if you don’t have:

• A history of cancer.
• Acute liver failure.
• Acute respiratory failure.
• Ascites.
• Varices.

What is the life expectancy with Budd-Chiari?

Without treatment, most people with a completely blocked hepatic vein die of liver failure within three to five years. You can live longer with a liver transplant. Research shows people also usually live longer if their veins are only partially blocked or narrowed.

Your healthcare provider is your best resource for explaining your prognosis (likely outcomes) based on your specific diagnosis and health.

How do I take care of myself?

Follow your healthcare provider’s instructions on what follow-up care you’ll need to monitor your liver. You may need:

• Frequent blood tests.
• Regular imaging procedures or biopsies.
• Close follow-up with a liver specialist (hepatologist).
• Ongoing treatment to control ascites (including a sodium-restricted diet, diuretics or a procedure called paracentesis, which drains excess fluid from your belly).

What questions should I ask my healthcare provider?

Questions to ask include:

• Do I have an underlying condition that caused Budd-Chiari syndrome?
• Do I have liver damage?
• Do I have cirrhosis (end-stage scarring of the liver)?
• Am I at risk of developing a complication?
• What treatments do you recommend?
• What specialists will I need to see during my treatment?
• What type of follow-up care will I need?