Budd-Chiari Syndrome During Pregnancy and Its Impact on the Mother and Fetus

Budd–Chiari syndrome is a rare vascular disorder characterized by obstruction of hepatic venous outflow, leading to liver congestion and, in severe cases, potentially progressing to impaired liver function. This syndrome is of particular importance during pregnancy due to the physiological changes that occur during this period, especially the increased tendency of blood to clot, which may contribute to a higher risk of venous thrombosis or exacerbate the condition in affected women.Pregnancy in women with Budd–Chiari syndrome presents a medical challenge that requires close monitoring and a multidisciplinary approach involving hepatology, hematology, and obstetrics specialists. The potential risks are not limited to maternal health but may also affect pregnancy outcomes and fetal well-being. These risks may include an increased likelihood of preterm birth or intrauterine growth restriction in some cases, particularly when hepatic complications or portal hypertension are present. However, the impact of the syndrome on the fetus is not inevitable, as it depends on disease severity, the stability of liver function, the presence of associated complications, and the quality of medical follow-up and appropriate treatment.Therefore, at Medica Guide, it is important to investigate the relationship between Budd–Chiari syndrome and pregnancy, with a particular focus on its potential effects on the fetus, the factors that may influence pregnancy outcomes, and the role of early diagnosis and specialized monitoring in reducing complications and improving the likelihood of favorable maternal and fetal outcomes.

What Is Pregnancy-Associated Budd–Chiari Syndrome?

Budd–Chiari syndrome is a rare condition characterized by obstruction of the hepatic veins, the blood vessels responsible for draining blood from the liver. This obstruction is often caused by the formation of blood clots. The syndrome may develop during pregnancy or in the postpartum period due to the increased tendency of blood to clot during these stages.

Does Budd–Chiari Syndrome Affect the Fetus?

Budd–Chiari syndrome may be associated with an increased risk of certain pregnancy complications, such as preterm birth, cesarean delivery, or the need for other obstetric interventions, particularly when the condition is unstable or accompanied by hepatic or vascular complications. However, limited studies have shown that pregnancy outcomes can be favorable in women whose condition is stable before conception and who receive appropriate medical follow-up.

Does the Syndrome Increase the Risk of Miscarriage?

The risk of pregnancy loss may increase in some cases. However, accurately estimating this risk remains difficult because Budd–Chiari syndrome is rare and available studies are limited. The level of risk depends on the stability of the condition, the presence of associated blood clotting disorders, the development of complications during pregnancy, and other factors related to maternal and fetal health.

Can Women with Budd–Chiari Syndrome Have a Vaginal Delivery?

Yes. Vaginal delivery may be an appropriate option for some affected women, and Budd–Chiari syndrome alone is not an automatic indication for cesarean delivery. The mode of delivery is determined according to the health of the mother and fetus, the stability of liver function, and the presence of complications such as ascites or varices. Other important considerations include the type and dose of anticoagulant medication used and the timing of its temporary discontinuation before delivery, while also taking into account the requirements for spinal or epidural anesthesia.

What Happens After Delivery?

The postpartum period is an important stage in the care of women with Budd–Chiari syndrome because the risk of blood clots remains elevated during this time. The patient may need to resume anticoagulant therapy after delivery, depending on an assessment of bleeding risk and the recommendations of the medical

This period also requires monitoring of liver function, assessment for ascites and other complications, and surveillance for recurrent thrombosis. In addition, the treatment plan should be reviewed to determine whether it is compatible with breastfeeding.

Is Breastfeeding Possible with Budd–Chiari Syndrome?

Breastfeeding may be possible for women with Budd–Chiari syndrome, but this depends on the type of medication being used and its safety during lactation. According to recommendations in the guidelines of the European Association for the Study of the Liver (EASL), warfarin can be used during breastfeeding, whereas direct oral anticoagulants are not recommended during this period under those guidelines.

The appropriate treatment should be determined in consultation with a specialist, taking into account the mother's health condition and therapeutic needs.

Does Pregnancy Cause Budd–Chiari Syndrome?

Not necessarily. Pregnancy is not the sole cause of Budd–Chiari syndrome, but it is a condition in which the blood becomes more prone to clotting. Pregnancy and the postpartum period may contribute to the development of the syndrome in women who have a pre-existing predisposition to thrombosis, whether due to inherited or acquired clotting disorders or other risk factors.

The syndrome may be diagnosed during pregnancy or in the postpartum period, which is a particularly important time for monitoring thrombotic complications.

Causes of Pregnancy-Associated Budd–Chiari Syndrome

Budd–Chiari syndrome is a rare condition caused by impaired venous blood flow out of the liver. This is most commonly due to the formation of a blood clot in the hepatic veins, although the obstruction may also extend to the inferior vena cava.

Pregnancy is associated with this syndrome because of the physiological changes that occur in the body and increase the tendency of blood to clot, particularly during the final months of pregnancy and the postpartum period. However, pregnancy is not the only cause of the condition; rather, it may contribute to the development of the syndrome in women who already have a predisposition to blood clot formation.

What Are the Pregnancy-Related Causes and Risk Factors?

1. Increased Blood Clotting Tendency During Pregnancy

During pregnancy, natural changes occur in the blood coagulation system, including increased levels of certain clotting factors and reduced activity of some of the body's natural anticoagulant mechanisms. These changes increase the likelihood of blood clot formation, including clots that may affect the hepatic veins.

2. The Postpartum Period

The postpartum period is an important stage during which the risk of blood clots is elevated, as pregnancy-related changes in the coagulation system persist after delivery. Consequently, Budd–Chiari syndrome may develop during this period, even if no symptoms were present during pregnancy.

3. Inherited Blood Clotting Disorders

Certain inherited disorders can increase the risk of blood clot formation. The most notable include:

Factor V Leiden mutation.
Prothrombin gene mutation.
Protein C deficiency.

Protein S deficiency.

Antithrombin deficiency.

The presence of any of these disorders, combined with the physiological changes associated with pregnancy, may increase the risk of venous thrombosis.

4. Blood Disorders Associated with Increased Blood Cell Production

Certain blood disorders are associated with an increased risk of Budd–Chiari syndrome, particularly myeloproliferative neoplasms, such as polycythemia vera and myelofibrosis. The JAK2 gene mutation may be present in some affected women, even when an elevated blood cell count is not evident in initial laboratory tests.

5. Antiphospholipid Syndrome

Antiphospholipid syndrome (APS) is an autoimmune disorder that increases the risk of blood clot formation. It may also be associated with pregnancy complications, making it an important risk factor to evaluate in some women with Budd–Chiari syndrome.

6. Other Factors That May Increase the Risk

Additional factors may contribute to an increased likelihood of blood clot formation, particularly when combined with pregnancy-related changes. These include:

Severe dehydration.
Certain infections.
Obesity.
Prolonged periods of immobility.
A previous history of blood clots.

Symptms of Pregnancy-Associated Budd–Chiari Syndrome

The symptoms of Budd–Chiari syndrome during pregnancy vary from one woman to another, depending on the degree of hepatic vein obstruction, how rapidly it develops, and the extent to which liver function is affected. Symptoms may appear suddenly and acutely or develop gradually over weeks or months. Diagnosing the syndrome during pregnancy can be challenging because some symptoms may resemble normal pregnancy symptoms or those of other conditions requiring urgent medical intervention.

1. Pain in the Upper Right Abdomen

Abdominal pain, particularly in the upper right quadrant beneath the ribs, is a common symptom of the syndrome. It is usually caused by liver congestion and enlargement resulting from impaired hepatic venous drainage.

The pain may present as:

Pain beneath the right ribs or in the middle of the upper abdomen.
Persistent or progressively worsening pain.
A sensation of pressure or fullness in the abdomen.
Pain accompanied by nausea or vomiting.

In acute cases, the pain may be severe and develop over a short period. However, abdominal pain alone is insufficient to diagnose the syndrome, as it may also occur with gallbladder disease, preeclampsia, or other conditions.

2. Hepatomegaly (Enlarged Liver)

The liver enlarges when blood cannot drain from it normally, resulting in blood accumulation and an increase in liver size.

Associated symptoms may include:

Pain or tenderness when pressure is applied to the upper right abdomen.
A sensation of fullness or heaviness after eating.
Abdominal discomfort or bloating.

A physician may suspect hepatomegaly during a physical examination and confirm it using ultrasound imaging. Doppler ultrasound may also be used to assess blood flow through the hepatic veins.

3. Ascites: Fluid Accumulation in the Abdomen

Ascites is an important sign of Budd–Chiari syndrome and may be one of the first symptoms that prompts a patient to seek medical attention. It develops due to increased pressure in the hepatic veins and portal venous system, causing fluid to leak and accumulate within the abdominal cavity.

Common signs include:

A noticeable increase in abdominal circumference.
Rapid weight gain due to fluid retention.
A sensation of abdominal heaviness or pressure.
Early satiety and reduced appetite.
Shortness of breath when fluid accumulation is substantial

During pregnancy, it may be difficult to distinguish normal abdominal enlargement from ascites. Therefore, a physician may use ultrasound to identify fluid accumulation and assess the condition of the liver

4. Swelling of the Legs and Feet

Leg swelling may occur due to fluid retention or impaired venous circulation, although it is not specific to Budd–Chiari syndrome.

During pregnancy, it is important to distinguish gradual leg swelling, which may occur normally, from swelling that requires medical evaluation.

Sudden or severe swelling, particularly when accompanied by high blood pressure, a severe headache, or visual disturbances, requires urgent assessment for possible pregnancy complications, including preeclampsia.

Swelling in one leg accompanied by pain, redness, or warmth may indicate deep vein thrombosis and requires urgent medical evaluation.

5. Nausea, Vomiting, and Loss of Appetite

Patients may experience nausea, vomiting, and loss of appetite due to liver congestion or impaired liver function. However, these symptoms are nonspecific, particularly during the first trimester, when they may be part of normal pregnancy.

These symptoms become more concerning when they are severe or progressively worsening, or when accompanied by severe upper abdominal pain, yellowing of the eyes, a rapid increase in abdominal size, or reduced urine output.

6. Jaundice: Yellowing of the Skin and Eyes

Jaundice may develop when the liver's ability to process and eliminate bilirubin is impaired, potentially indicating reduced liver function.

Signs include:

Yellowing of the whites of the eyes and the skin.
Dark-colored urine.
Itching in some cases, particularly when an associated disorder of bile flow is present.

Jaundice does not occur in all patients with Budd–Chiari syndrome. It may be absent in the early stages or in cases where liver function remains relatively preserved.

7. Fatigue and General Weakness

Patients may experience unusual fatigue or general weakness that interferes with daily activities. This may be associated with impaired liver function, poor nutrition, or accompanying complications.

However, fatigue is common during pregnancy and is not, by itself, evidence of Budd–Chiari syndrome. It should be assessed in the context of other symptoms and medical test results.

8. Signs of Portal Hypertension

Persistent hepatic vein obstruction may lead to increased pressure within the portal venous system, increasing the risk of complications, including:

Splenomegaly (enlarged spleen).
Dilated veins in the esophagus or stomach, known as esophageal or gastric varices.
A reduced platelet count in some cases due to splenic enlargement and platelet sequestration.
Gastrointestinal bleeding, which may present as vomiting blood or passing black stools.

Vomiting blood or passing black stools are serious warning signs that require immediate hospital evaluation.

9. Signs of Acute Liver Failure

In severe cases, Budd–Chiari syndrome may progress to acute liver failure, particularly when the obstruction is extensive or develops suddenly.

Warning signs may include:

Altered consciousness or confusion.
Easy bleeding or bruising.
Rapid deterioration in general health.
Significant abnormalities in liver function and blood clotting tests

Medical Treatment of Pregnancy-Associated Budd–Chiari Syndrome

Treatment of Budd–Chiari syndrome during pregnancy focuses on preventing the extension of existing blood clots, reducing the formation of new clots, improving hepatic venous blood flow, and managing associated complications, such as ascites and portal hypertension. Treatment requires careful assessment of disease severity, liver and kidney function, and gestational age, while prioritizing the safety of both the mother and fetus.

Anticoagulants are a cornerstone of treatment. Low-molecular-weight heparin (LMWH) is generally used during pregnancy because it does not cross the placenta in clinically significant amounts. Given the seriousness of this syndrome and the potential for complications, treatment should be supervised by a multidisciplinary team that includes hepatologists, hematologists, and obstetricians.

First: Essential Medications for Treating Budd–Chiari Syndrome

1. Low-Molecular-Weight Heparin (LMWH)

Low-molecular-weight heparin is one of the preferred anticoagulants during pregnancy. Examples include:

Enoxaparin.
Dalteparin.

Mechanism of Action and Use

This type of medication helps prevent existing blood clots from growing and reduces the formation of new clots, thereby lowering the risk of worsening venous obstruction.

Low-molecular-weight heparin is preferred during pregnancy because it does not cross the placenta in clinically significant amounts and is therefore not expected to directly affect fetal blood coagulation.

Necessary Precautions

The dose is determined according to the patient's weight, kidney function, and disease severity, with a distinction between therapeutic and prophylactic doses.
Blood counts, platelet levels, and kidney function should be assessed, and signs of bleeding should be monitored throughout treatment.
The timing of doses must be coordinated with the medical team as delivery approaches, particularly when spinal or epidural anesthesia is planned.
Heparin doses must not be prescribed or adjusted without specialist medical assessment.

2. Warfarin and Direct Oral Anticoagulants

The suitability of anticoagulants varies depending on whether the patient is pregnant or in the postpartum period. Treatment must therefore be selected according to each patient's clinical circumstances.

Warfarin: Warfarin is generally avoided during pregnancy because of its potential risks to the fetus, particularly during early pregnancy. It may be used in selected cases after delivery under medical supervision and is considered compatible with breastfeeding according to medical recommendations.

Direct oral anticoagulants (DOACs), such as rivaroxaban and apixaban: These medications are not recommended during pregnancy because of limited safety data and potential fetal risks. They are also generally not recommended during breastfeeding.

After delivery, the anticoagulation plan and treatment duration should be reassessed. Women with Budd–Chiari syndrome often require long-term anticoagulation, potentially lifelong, depending on the underlying cause of thrombosis, the risk of recurrence, and the presence of contraindications to treatment.

Second: Medications Used to Treat Complications

Additional medications may be required to control complications resulting from liver congestion or portal hypertension. The choice of medication depends on the patient's clinical condition, gestational age, and liver and kidney function.

Complication	Potential medical treatment	Important considerations
Ascites	Diuretics, such as furosemide, in selected cases	Kidney function, electrolyte levels, and maternal and fetal well-being require careful monitoring.
Esophageal varices and portal hypertension	Beta-blockers, such as propranolol, in selected cases	Maternal blood pressure and fetal growth must be monitored. Endoscopic variceal ligation may be necessary.
Hepatic encephalopathy	Lactulose when indicated	Used to reduce ammonia absorption and manage encephalopathy, not to treat the underlying blood clot.
Coagulation disorders or severe liver failure	Cause-specific treatment in a hospital setting	Blood products or plasma should not be administered routinely without a specific medical indication.

1. Diuretics for Ascites

Furosemide may be used in selected cases to reduce fluid retention. However, its use during pregnancy requires caution and careful monitoring of fluid balance, electrolyte levels, and kidney function.

Spironolactone, which is commonly used to treat ascites associated with liver disease, is generally avoided during pregnancy because of concerns about its antiandrogenic effects and potential impact on fetal development.

In cases of severe ascites, additional hospital-based interventions may be necessary, depending on the underlying cause and severity of symptoms.

2. Beta-Blockers for Esophageal Varices

Propranolol may be used in selected cases to reduce the risk of bleeding from esophageal varices caused by portal hypertension.

The physician must weigh the expected benefits against potential risks while monitoring maternal blood pressure and heart rate and assessing fetal growth. Endoscopic variceal ligation may also be an appropriate option in some cases, depending on the clinical assessment.

3. Lactulose for Hepatic Encephalopathy

Lactulose may be used if the condition progresses to hepatic encephalopathy, a disorder that can cause confusion or altered consciousness due to impaired liver function.

Treatment aims to reduce ammonia absorption from the intestines and improve symptoms. However, lactulose does not treat the obstruction of the hepatic veins itself.

Third: Are Medications Alone Sufficient to Treat Budd–Chiari Syndrome

Medications alone are not sufficient in every case. Although anticoagulants are essential, some patients may require additional interventions if the obstruction persists, liver function deteriorates, or serious complications develop.

Depending on the clinical condition, treatment options may include:

Thrombolysis or mechanical removal of the obstruction in selected cases.
Balloon angioplasty or stent placement when necessary.
Transjugular intrahepatic portosystemic shunt (TIPS) placement to improve blood drainage in suitable cases.
Liver transplantation in severe cases that do not respond to other treatments.

The timing and suitability of these interventions during pregnancy must be determined by a specialist team, balancing the need to treat the mother against fetal safety.

Fourth: Important Precautions During Pregnancy and After Delivery

Women with Budd–Chiari syndrome require regular follow-up, including assessment of liver and kidney function, monitoring of blood counts and platelet levels, evaluation of bleeding and thrombosis risks, and surveillance of fetal growth and well-being.

The postpartum period is particularly important because the risk of blood clots remains elevated after delivery. A clear plan should therefore be established for restarting anticoagulation and determining treatment duration in coordination with the medical team.

Surgical Treatment of Pregnancy-Associated Budd–Chiari Syndrome

Budd–Chiari syndrome is a rare vascular disorder caused by obstruction of hepatic venous outflow, most commonly due to blood clots in the hepatic veins. Treatment aims to restore blood flow out of the liver, reduce complications associated with hepatic congestion and portal hypertension, and protect the health of both the mother and fetus

Not all cases require surgical intervention. Treatment usually begins with anticoagulants, while interventional or surgical procedures are considered when medication does not achieve the desired results or the severity of the condition requires additional treatment. The choice of procedure depends on the location and extent of the obstruction, the degree of liver dysfunction, gestational age, and the mother's overall health.

First: The Standard Treatment Sequence

The main treatment options can be summarized as follows:

Medical treatment and anticoagulation: Used in most cases to prevent existing clots from extending and new clots from forming.
Balloon angioplasty with stent placement when necessary: Suitable for certain cases involving short-segment stenosis or obstruction that can be reopened.
Transjugular intrahepatic portosystemic shunt (TIPS): Considered when previous treatment is insufficient or unsuitable.
Liver transplantation: Considered in cases of acute liver failure or deterioration despite other treatments.

This sequence is a general guide rather than a fixed treatment plan. Some cases may require urgent intervention without progressing through every stage.

Second: Interventional and Surgical Treatment Methods

1. Balloon Angioplasty of the Hepatic Veins

When is it used?

This procedure is suitable when the obstruction results from a short or localized narrowing in a hepatic vein or the inferior vena cava that can be reopened without creating an alternative venous pathway.

How is it performed?

The location and extent of the obstruction are assessed using Doppler ultrasound, with additional imaging if necessary.
A catheter is inserted into a blood vessel, usually through the femoral or jugular vein, depending on the patient's vascular anatomy.
The catheter is guided to the narrowed area, and imaging is used to identify the obstruction and assess blood flow.
A guidewire is passed through the narrowed segment, and a medical balloon is inflated to widen it.
Blood flow is reassessed, and a stent may be inserted if narrowing persists or the risk of recurrence is high.

Risks and precautions during pregnancy

Potential complications include bleeding, blood vessel injury, and recurrent obstruction. The procedure requires minimizing exposure to ionizing radiation and using the lowest appropriate dose of contrast material, in coordination with interventional radiologists and obstetric specialists.