What Is Budd-Chiari Syndrome? Symptoms, Causes, and Treatment Options
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick Answer: What is Budd-Chiari syndrome?
Budd-Chiari syndrome is a blockage or narrowing of the veins that carry blood away from the liver, causing an enlarged liver and spleen, abdominal pain, fluid buildup, jaundice and fatigue. [4] It is most often linked to conditions that increase clotting. [1] Treatment uses blood thinners, stents, shunts or balloon procedures, with liver transplant for severe cases. [4] Prompt treatment is essential. [4]
Key takeaways
Budd-Chiari syndrome is a narrowing or blockage of the hepatic veins that drain blood away from the liver. [4]
Blood backs up into the liver, enlarging the liver and sometimes the spleen, and can trigger portal hypertension, varices, ascites, and cirrhosis. [4]
The most common form is subacute, developing gradually over several months; 15% to 20% of subacute and chronic cases have no symptoms. [4]
Common causes are clotting-related: blood cancers (MPNs), hypercoagulable disorders, sickle cell disease, IBD, pregnancy, and oral contraceptives. One in five cases has no known cause. [4]
Doppler ultrasound is often the first — and sometimes only — test needed for diagnosis. [4]
Treatment restores blood flow with blood thinners, the TIPS shunt procedure, balloon angioplasty, or liver transplant in severe cases. [4]
What the research says
Most people with Budd-Chiari syndrome have a clotting risk factor, and survival is good with modern care. In a European series of 163 newly diagnosed patients, 84% had at least one thrombotic risk factor and 46% had more than one; myeloproliferative disorders were the most common (49% of 103 patients tested). With anticoagulation (86%), TIPS (34%), and liver transplantation (12%) used as needed, survival was 87% at 1 year and 82% at 2 years (Darwish Murad et al., Ann Intern Med, 2009) [1].
Opening the blocked veins with radiologic procedures can protect the liver. In 136 patients treated with hepatic vein and inferior vena cava recanalization or direct intrahepatic portosystemic shunt creation, transplant-free survival was 94% at both 1 year and 5 years, and in the recanalization group mean bilirubin fell from 1.8 to 1.4 mg/dL while albumin rose from 3.2 to 3.45 g/dL (Mukund et al., J Vasc Interv Radiol, 2018) [2].
TIPS helps many patients, but the shunt can narrow or block. A meta-analysis of 17 studies (618 patients) found 88% of patients alive between 1 and 10 years after TIPS (92% at five years and 77% at ten), only 6% needed a liver transplant despite TIPS, and 30% had shunt dysfunction (Moreno et al., Arq Gastroenterol, 2024) [3].
What Is Budd-Chiari Syndrome?
Budd-Chiari syndrome involves narrowing or a blockage (obstruction) in one or more of the veins that drain the liver — the hepatic veins. The narrowing can come from inflammation within the veins or from pressure (compression) outside the veins. An obstruction may block an entire vein or only part of it. The obstruction can be caused by a blood clot, but not always. [4]
The blockage can cause blood to back up into the liver, enlarging it. The spleen may also enlarge. [4]
What Complications Can It Cause?
Budd-Chiari syndrome may also cause other conditions, including:
Complication | What happens |
Portal hypertension | Increased pressure in the portal vein, which carries blood from the digestive tract and spleen to the liver |
Varices | Enlarged veins in the esophagus, stomach, or rectum |
Ascites | A buildup of fluid in the abdomen |
Cirrhosis | Irreversible scarring of the liver |
Types of Budd-Chiari Syndrome
Providers classify Budd-Chiari syndrome based on how long you have had it and the severity of your symptoms:
Type | How symptoms develop |
Acute with acute liver failure | Symptoms develop quickly (within days or weeks), and liver function rapidly declines |
Acute without liver failure | Symptoms develop quickly but without loss of liver function |
Subacute | Symptoms set in more slowly, usually over several months — the most common type |
Chronic | Symptoms usually aren't noticeable until the blockage has already caused irreversible scarring (cirrhosis) in the liver |
Primary vs. Secondary Budd-Chiari Syndrome
Type | Where the problem is | Common causes |
Primary | Narrowing or blockage within the blood vessels | Blood clot (bland thrombus), blockage from a collection of tumor cells, medical conditions causing inflammation of the hepatic veins |
Secondary | An issue outside the blood vessels | A malignant or benign tumor pressing on a hepatic vein or the inferior vena cava (IVC); conditions that injure liver cells or place pressure on hepatic veins, including certain fungal infections, trauma, or chemotherapy |
The inferior vena cava (IVC) is the large vein that drains blood from the hepatic veins into the right side of the heart. [4]
Budd-Chiari Syndrome Symptoms
The signs and symptoms of Budd-Chiari syndrome include:
Abdominal pain (particularly in the right upper abdomen).
Ascites (swelling in the 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 the esophagus, stomach, or rectum.
Edema (swelling) in the legs.
Fatigue (extreme tiredness).
Liver failure (liver no longer functions adequately). [4]
Can You Have No Symptoms at All?
The timing of symptoms depends on the type of Budd-Chiari syndrome. People with acute forms typically notice symptoms more quickly than people with subacute or chronic forms. Researchers estimate that 15% to 20% of people with subacute and chronic forms don't have symptoms. [4]
What Causes Budd-Chiari Syndrome?
Conditions and situations that lead to blood clots cause Budd-Chiari syndrome. These include:
Cause category | Details |
Myeloproliferative neoplasms (MPNs) | Rare blood cancers that cause the body to make too many blood cells; includes polycythemia vera, myelofibrosis, and essential thrombocytosis |
Hypercoagulable disorders | Conditions that cause excess clot formation; includes protein C or S deficiency, antiphospholipid antibody syndrome, antithrombin III deficiency, Factor V Leiden mutation, and lupus |
Masses in the liver or kidneys | Malignant or benign masses that can compress the 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 the digestive system |
Pregnancy | Increases the risk of blood clots, especially if you also have a condition that predisposes you to clots |
Oral contraceptives | Some forms of birth control pills increase the risk of blood clots |
One in five cases of Budd-Chiari syndrome is idiopathic — meaning healthcare providers can't determine a clear cause for the condition. [4]
How Is Budd-Chiari Syndrome Diagnosed?
A healthcare provider can diagnose Budd-Chiari syndrome through a physical exam and tests. They will ask about symptoms and look for signs like upper abdominal pain and swelling. They will arrange blood tests to check liver function and see whether you are at risk for blood clots. Up to 50% of people with Budd-Chiari syndrome have high liver enzyme levels. [4]
Imaging tests can show whether there is narrowing or obstruction of the hepatic veins or IVC:
Test | What it does |
Doppler ultrasound | Uses sound waves to record images of the liver, spleen, and kidneys; shows problems in blood vessels such as irregular blood flow; often the first and only test needed |
CT scan | Uses X-rays and computers to produce cross-section images; can show an enlarged liver and ascites; may require contrast material, and the provider may check kidney function first |
MRI | Uses a large magnet, radio waves, and a computer for very clear pictures; may also require contrast material |
Venography (venogram) | Uses X-rays to show blood flow in the veins; used when other tests haven't confirmed the diagnosis but the provider believes you likely have Budd-Chiari; invasive, requiring a contrast dye injection into the veins |
The provider — a radiologist or hepatologist — may also order a liver biopsy to see whether cirrhosis is present. [4] A sample of liver tissue is 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 the hepatic veins or IVC to decrease swelling and scar formation in the liver. Whenever possible, treatments involve managing the underlying condition that caused the clots. Managing complications, such as portal hypertension, is also essential. [4]
Medications
The healthcare provider may prescribe blood thinners (anticoagulants), such as heparin or warfarin, to dissolve the blood clots. [4]
Nonsurgical Procedures
You may need a procedure to help redirect blood flow and relieve pressure in the blood vessels. Procedures include:
Procedure | How it works |
TIPS (transjugular intrahepatic portosystemic shunt) | The provider makes a tunnel through the liver with a needle, connects the portal vein to one of the hepatic veins, and places a stent (tiny metal tube) in the tunnel — guided by X-ray imaging — to keep the track open |
Percutaneous transluminal angioplasty | A catheter (thin, hollow tube with a balloon tip) is inserted through the skin into a blood vessel and guided to the clot or narrowed area; the balloon is inflated to widen the vein, and a stent may be placed to keep it open |
Liver Transplant
You may need a liver transplant if other treatments haven't helped or aren't an option. For example, a liver transplant is likely needed if you have liver failure, cirrhosis of the liver, bleeding from varices, or ascites that can't be treated with medications. [4]
What Is the Outlook?
Prognosis
The prognosis depends on factors including how severe the condition is and the response to treatment. The outlook tends to be better if you don't have:
Factor | Effect on outlook |
History of cancer | Presence worsens outlook |
Acute liver failure | Presence worsens outlook |
Acute respiratory failure | Presence worsens outlook |
Ascites | Presence worsens outlook |
Varices | Presence worsens outlook |
Life Expectancy
Without treatment, most people with a completely blocked hepatic vein die of liver failure within three to five years. [4] You can live longer with a liver transplant. [4] Research shows people also usually live longer if their veins are only partially blocked or narrowed. [4]
The healthcare provider is the best resource for explaining the prognosis based on a specific diagnosis and health.
Living With Budd-Chiari Syndrome
How Do You Take Care of Yourself?
Follow the healthcare provider's instructions on follow-up care to monitor the liver. You may need frequent blood tests, regular imaging procedures or biopsies, close follow-up with a liver specialist (hepatologist), and ongoing treatment to control ascites — including a sodium-restricted diet, diuretics, or a procedure called paracentesis, which drains excess fluid from the belly. [4]
Questions to Ask Your Healthcare Provider
Question | Why it matters |
Do I have an underlying condition that caused Budd-Chiari syndrome? | Treatment may target the root cause |
Do I have liver damage? | Guides the treatment plan |
Do I have cirrhosis (end-stage scarring of the liver)? | Affects prognosis and options |
Am I at risk of developing a complication? | Helps with monitoring |
What treatments do you recommend? | Clarifies the plan |
What specialists will I need to see during my treatment? | Coordinates care |
What type of follow-up care will I need? | Ensures ongoing monitoring |
A note on urgency: Prompt treatment for Budd-Chiari syndrome is essential. [4] Whether through medications or a procedure like TIPS, the condition must be addressed so the hepatic veins can drain the liver. In severe cases, a liver transplant may be needed. Regardless of the treatment type, it's essential to follow guidance on follow-up care to treat the underlying condition and reduce the risk of complications.
Conclusion
Budd-Chiari syndrome is a serious but treatable condition in which the veins draining the liver become narrowed or blocked — most often by blood clots. When blood cannot leave the liver efficiently, it backs up, enlarging the liver and spleen and setting off a chain of complications: portal hypertension, enlarged veins, abdominal fluid buildup, and eventually irreversible scarring.
Because the condition is frequently tied to blood-clotting disorders, identifying and managing the underlying cause is a core part of treatment. Restoring blood flow — through anticoagulants, the TIPS shunt procedure, balloon angioplasty, or liver transplantation in severe cases — can dramatically change the outcome. Early diagnosis matters: subacute Budd-Chiari, the most common form, creeps in slowly over months, and up to a fifth of subacute and chronic cases carry no symptoms at all.
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Frequently Asked Questions
What is Budd-Chiari syndrome?
Budd-Chiari syndrome is a narrowing or blockage (obstruction) in one or more of the veins that drain the liver, called the hepatic veins.
What are the hepatic veins?
The hepatic veins are the three veins that carry blood away from the liver, draining it into the inferior vena cava, which delivers that blood to the right side of the heart.
What happens when hepatic veins are blocked?
Blood backs up into the liver, enlarging it. The spleen may also enlarge, and pressure builds in the portal vein system.
What complications can Budd-Chiari syndrome cause?
It can cause portal hypertension, varices (enlarged veins in the esophagus, stomach, or rectum), ascites (fluid buildup in the abdomen), and cirrhosis (irreversible liver scarring).
What is portal hypertension?
Portal hypertension is increased pressure in the portal vein, which carries blood from the digestive tract and spleen to the liver.
What are varices?
Varices are enlarged veins that can develop in the esophagus, stomach, or rectum when blood flow through the liver is obstructed.
What is ascites?
Ascites is a buildup of fluid in the abdomen, causing abdominal swelling.
What is cirrhosis?
Cirrhosis is irreversible scarring of the liver.
What causes the narrowing in Budd-Chiari syndrome?
The narrowing can be from inflammation within the veins or pressure (compression) outside the veins.
Is Budd-Chiari syndrome always caused by a blood clot?
No. The obstruction can be from a blood clot, but not always — tumors, compression, or inflammation can also cause it.
How many types of Budd-Chiari syndrome are there?
Providers classify it into four types based on timing and severity: acute with liver failure, acute without liver failure, subacute, and chronic.
What is acute Budd-Chiari syndrome?
Acute Budd-Chiari syndrome develops quickly, within days or weeks. It can occur with acute liver failure (rapidly declining liver function) or without loss of liver function.
What is subacute Budd-Chiari syndrome?
Subacute Budd-Chiari syndrome develops more slowly, usually over several months. It is the most common type.
What is chronic Budd-Chiari syndrome?
Chronic Budd-Chiari syndrome typically has no noticeable symptoms until the blockage has already caused irreversible scarring (cirrhosis) in the liver.
What is the difference between primary and secondary Budd-Chiari syndrome?
Primary Budd-Chiari occurs because of a narrowing or blockage within the blood vessels (such as a blood clot or tumor cell collection). Secondary Budd-Chiari occurs because of an issue outside the blood vessels, such as a tumor pressing on a hepatic vein or the inferior vena cava.
What is the inferior vena cava?
The inferior vena cava (IVC) is the large vein that drains blood from the hepatic veins into the right side of the heart.
What are myeloproliferative neoplasms (MPNs)?
MPNs are rare blood cancers that cause the body to make too many blood cells. They include polycythemia vera, myelofibrosis, and essential thrombocytosis.
What are hypercoagulable disorders?
Hypercoagulable disorders are conditions that cause excess clot formation. They include protein C or S deficiency, antiphospholipid antibody syndrome, antithrombin III deficiency, Factor V Leiden mutation, and lupus.
Can pregnancy cause Budd-Chiari syndrome?
Pregnancy increases the risk of blood clots, especially if you also have a condition that predisposes you to clotting.
Can birth control pills cause Budd-Chiari syndrome?
Some forms of oral contraceptives increase the risk of blood clots, which can contribute to Budd-Chiari syndrome.
What does idiopathic mean?
Idiopathic means healthcare providers can't determine a clear cause. One in five cases of Budd-Chiari syndrome is idiopathic.
What are the symptoms of Budd-Chiari syndrome?
Symptoms include right upper abdominal pain, ascites (abdominal swelling from fluid), jaundice, hepatic encephalopathy (confusion or coma), enlarged liver, enlarged spleen, bleeding from the esophagus, stomach, or rectum, leg swelling, extreme fatigue, and liver failure.
What is hepatic encephalopathy?
Hepatic encephalopathy is confusion or coma caused by liver disease.
What is hepatomegaly?
Hepatomegaly is an enlarged liver.
What is splenomegaly?
Splenomegaly is an enlarged spleen.
What is edema?
Edema is swelling, such as the swelling in the legs seen in Budd-Chiari syndrome.
Can you have Budd-Chiari syndrome without symptoms?
Yes. Researchers estimate that 15% to 20% of people with subacute and chronic forms don't have symptoms.
How is Budd-Chiari syndrome diagnosed?
It is diagnosed through a physical exam, blood tests checking liver function and clot risk, and imaging tests such as Doppler ultrasound, CT scan, MRI, or venography. A liver biopsy may also be ordered.
What is a Doppler ultrasound?
Doppler ultrasound uses sound waves to record images of the liver, spleen, and kidneys. It can show irregular blood flow in the veins and is often the first — and sometimes the only — test needed.
What is a venogram?
A venogram (venography) uses X-rays to show blood flow in the veins. It is invasive and involves injecting contrast dye into the veins.
Can liver enzymes be elevated in Budd-Chiari syndrome?
Yes. Up to 50% of people with Budd-Chiari syndrome have high liver enzyme levels.
How is Budd-Chiari syndrome treated?
Treatment aims to dissolve clots or improve narrowing in the hepatic veins or IVC using blood thinners, minimally invasive procedures (TIPS, balloon angioplasty), and, in severe cases, liver transplantation. Managing the underlying cause and complications like portal hypertension is also essential.
What blood thinners are used?
Providers may prescribe anticoagulants such as heparin or warfarin to help dissolve blood clots.
What is the TIPS procedure?
TIPS (transjugular intrahepatic portosystemic shunt) is a procedure where a provider makes a tunnel through the liver with a needle, connects the portal vein to a hepatic vein, and places a stent (tiny metal tube) to keep the track open, using X-ray guidance.
What is percutaneous transluminal angioplasty?
It is a procedure in which a catheter with a balloon tip is guided through the skin into a blood vessel to the narrowed area; the balloon is inflated to widen the vein, and a stent may be placed to keep it open.
When is a liver transplant needed?
A liver transplant may be needed if other treatments haven't helped, or if there is liver failure, cirrhosis, bleeding from varices, or ascites that can't be treated with medications.
What is the life expectancy with Budd-Chiari syndrome?
Without treatment, most people with a completely blocked hepatic vein die of liver failure within three to five years. Life expectancy is longer with a liver transplant or when veins are only partially blocked or narrowed.
What improves the prognosis?
The outlook is better without a history of cancer, acute liver failure, acute respiratory failure, ascites, or varices.
How do I take care of myself after diagnosis?
Follow your provider's follow-up plan: frequent blood tests, regular imaging or biopsies, close follow-up with a hepatologist, and ongoing ascites treatment with a sodium-restricted diet, diuretics, or paracentesis (draining excess belly fluid).
What is paracentesis?
Paracentesis is a procedure that drains excess fluid from the belly to control ascites.
What should I do now?
Prompt treatment is essential — seek care from a healthcare provider so the hepatic veins can drain the liver, the underlying condition can be treated, and the risk of complications can be reduced.
Related Reading
Understanding the Hepatic Veins: Anatomy, Function, and Health
Blood Clotting Disorders: Causes, Symptoms, and Treatment Explained
Portal Hypertension: Complete Guide to Symptoms, Causes, Diagnosis, and Treatment
Hepatic Encephalopathy: Symptoms, Causes, and Treatment Explained
Acute Liver Failure (Fulminant Hepatic Failure): Symptoms, Causes, and Emergency Treatment
Polycythemia Vera: Complete Guide to Symptoms, Causes, Diagnosis, and Treatment
Myelofibrosis Complete Guide: Symptoms, Causes, Diagnosis, Treatment & Outlook
Sickle Cell Disease: Causes, Symptoms, and Modern Treatment Options
Understanding the Liver Biopsy Procedure: Types, Risks, and Benefits
TIPS Procedure: Transjugular Intrahepatic Portosystemic Shunt
References
Darwish Murad S, Plessier A, Hernandez-Guerra M, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009;151(3):167-175. PMID: 19652186. https://doi.org/10.7326/0003-4819-151-3-200908040-00004
Mukund A, Mittal K, Mondal A, et al. Anatomic recanalization of hepatic vein and inferior vena cava versus direct intrahepatic portosystemic shunt creation in Budd-Chiari syndrome: overall outcome and midterm transplant-free survival. J Vasc Interv Radiol. 2018;29(6):790-799. PMID: 29705227. https://doi.org/10.1016/j.jvir.2018.01.781
Moreno MOA, Paz CLDSL, Dezan MGF, et al. Ten-year outcomes of TIPS for Budd-Chiari syndrome: systematic review and meta-analysis. Arq Gastroenterol. 2024;61:e23171. PMID: 38896573. https://doi.org/10.1590/S0004-2803.24612023-171
Cleveland Clinic. Budd-Chiari Syndrome. Cleveland Clinic. Last updated February 4, 2024. https://my.clevelandclinic.org/health/diseases/21097-budd-chiari-syndrome
Medical disclaimer
This article is for general informational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of a qualified healthcare provider with any questions about a medical condition. Symptoms such as unexplained abdominal pain, abdominal swelling, jaundice, or leg swelling should be evaluated by a healthcare professional promptly.
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