Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

primary: non-fulminant

1st line – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
asymptomatic
1st line – 

anticoagulation

Asymptomatic patients require anticoagulation (unless there are contraindications) as underlying prothrombotic states are often present.

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with Budd-Chiari syndrome (BCS). Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.[1]

The improved prognosis of BCS is associated with the generalised use of anticoagulation, including in asymptomatic patients.[38]

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
asymptomatic
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

1st line – thrombolysis

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
1st line – 

thrombolysis

Thrombolysis may be used to relieve hepatic venous outflow obstruction and restore normal blood flow.[42] It is most effective in acute Budd-Chiari syndrome within 72 hours.

Local, systemic, or combined thrombolysis has been used.[56]

Localised thrombolytic therapy can be administered when a clot is fresh, by infusing the thrombolytic agent directly into the thrombosed vein.

Consider – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42] 

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with Budd-Chiari syndrome. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – symptom control

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

symptom control

Additional treatment recommended for SOME patients in selected patient group

Ascites is treated with diuretics (e.g., furosemide, spironolactone). Combined diuretic therapy is recommended if response to single therapy is not satisfactory. If ascites is still not controlled by medical therapy, paracentesis may be required.

History of bleeding due to portal hypertension is managed with pharmacological or endoscopic therapy.[38]

Primary options

furosemide: 20-40 mg orally once daily

OR

spironolactone: 50-200 mg orally once daily

Secondary options

furosemide: 20-40 mg orally once daily

and

spironolactone: 50-200 mg orally once daily

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

2nd line – radiological interventional therapy

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
2nd line – 

radiological interventional therapy

Hepatic angioplasty (balloon dilation with or without stent insertion) of localised narrowed hepatic veins is reported to relieve symptoms in 70% of patients.[15][58] However, success rates vary widely across centres.

Hepatic venographic approaches include a femoral or transjugular route, direct percutaneous transhepatic approach, or a combined approach. A combined approach is used when ultrasound shows patency of a significant segment of a major hepatic vein, but this is inaccessible from the inferior vena cava (IVC).[59][60]

IVC balloon dilation is usually sufficient to open up the obstruction (web or stenosis). IVC stent insertion is usually needed when balloon dilation is insufficient alone, or when there is recoil, or recurrent stenosis.[63]

Transjugular intrahepatic portosystemic shunt (TIPS) is considered the most common interventional procedure for Budd-Chiari syndrome in Western countries.[66] For the shunt to function, the portacaval pressure gradient should be <10 mmHg.

Failure of angioplasty (because the remaining patent veins are too small or have insufficient flow) and the presence of diffuse hepatic vein thrombosis are indications for TIPS.

Elective TIPS may be performed in patients with refractory ascites, recurrent variceal bleeding, hepatic outflow obstruction resulting from compression of the intrahepatic IVC by a hypertrophied caudate lobe, or poor hepatic reserve precluding surgical shunting.

Consider – localised thrombolysis

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

localised thrombolysis

Additional treatment recommended for SOME patients in selected patient group

When a clot is fresh, thrombolytic therapy can be given alongside interventional treatment, by infusing a thrombolytic agent directly into the involved vein.

Where there is venous obstruction of any cause and thrombus, localised thrombolytic therapy can be administered (via a catheter), prior to balloon dilatation or stenting of the obstructed vessel.[76]

Consider – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42] 

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with Budd-Chiari syndrome. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – symptom control

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

symptom control

Additional treatment recommended for SOME patients in selected patient group

Ascites is treated with diuretics (e.g., furosemide, spironolactone). Combined diuretic therapy is recommended if response to single therapy is not satisfactory. If ascites is still not controlled by medical therapy, paracentesis may be required.

History of bleeding due to portal hypertension is managed with pharmacological or endoscopic therapy.[38]

Primary options

furosemide: 20-40 mg orally once daily

OR

spironolactone: 50-200 mg orally once daily

Secondary options

furosemide: 20-40 mg orally once daily

and

spironolactone: 50-200 mg orally once daily

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

3rd line – surgical shunting

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
3rd line – 

surgical shunting

Indicated in patients not improving by radiological angioplasty and if the portacaval venous pressure gradient is ≥10 mmHg.

Indicated if there is good hepatic reserve and insignificant fibrosis. Better surgical outcomes are seen in patients with Child-Pugh class A and where the underlying cause has a favourable long-term outcome, such as essential thrombocythaemia.[42]

Side-to-side portacaval shunts, side-to-side mesocaval shunts, and cavoatrial shunts are used for patients with both caval and hepatic venous obstruction.[71]

Mesoatrial shunt is indicated if the inferior vena cava is obstructed, thrombosed, or compressed by a hypertrophied caudate lobe, resulting in low portacaval pressure gradient (however, a transjugular intrahepatic portosystemic shunt is usually a better approach in this setting).

Inferior vena caval membranous obstruction is managed by transatrial membranectomy, or, less commonly, by dorsocranial resection of the liver with hepatico-atrial anastomosis.[71][72]

Consider – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42]

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with Budd-Chiari syndrome. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – symptom control

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

symptom control

Additional treatment recommended for SOME patients in selected patient group

Ascites is treated with diuretics (e.g., furosemide, spironolactone). Combined diuretic therapy is recommended if response to single therapy is not satisfactory. If ascites is still not controlled by medical therapy, paracentesis may be required.

History of bleeding due to portal hypertension is managed with pharmacological or endoscopic therapy.[38]

Primary options

furosemide: 20-40 mg orally once daily

OR

spironolactone: 50-200 mg orally once daily

Secondary options

furosemide: 20-40 mg orally once daily

and

spironolactone: 50-200 mg orally once daily

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

4th line – liver transplant

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
4th line – 

liver transplant

Indications for transplantation include advanced cirrhosis, and failure of non-surgical treatment approaches or portosystemic shunting.[38][73][74]

Seventy-five percent of patients with Budd-Chiari syndrome (BCS) have a detectable genetic hypercoagulable state. Liver transplantation can cure almost all hereditary thrombophilias; however, thrombosis can still occur and anticoagulation is necessary.[4]

Living-donor liver transplantation using modified cavoplasty is a highly effective treatment for BCS and, in conjunction with long-term anticoagulant therapy and interventional radiological treatment, provides good long-term survival.[75]

Consider – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Most patients with Budd-Chiari syndrome (BCS) exhibit important risk factors for thrombosis; thus anticoagulation is best continued after transplantation.[38]

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42]

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with BCS. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: <72 hours from symptom onset or fresh clot seen on venography
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

1st line – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
1st line – 

anticoagulation

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42]

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with Budd-Chiari syndrome. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – symptom control

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

symptom control

Additional treatment recommended for SOME patients in selected patient group

Ascites is treated with diuretics (furosemide, spironolactone). Combined diuretic therapy is recommended if response to single therapy is not satisfactory. If ascites is still not controlled by medical therapy, paracentesis may be required.

History of bleeding due to portal hypertension is managed with pharmacological or endoscopic therapy.[38]

Primary options

furosemide: 20-40 mg orally once daily

OR

spironolactone: 50-200 mg orally once daily

Secondary options

furosemide: 20-40 mg orally once daily

and

spironolactone: 50-200 mg orally once daily

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

2nd line – radiological interventional therapy

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
2nd line – 

radiological interventional therapy

Hepatic angioplasty (balloon dilation with or without stent insertion) of localised narrowed hepatic vein is reported to relieve symptoms in 70% of patients.[15][58] However, success rates vary widely across centres.

Hepatic venographic approaches include a femoral or transjugular route, direct percutaneous transhepatic approach, or a combined approach. A combined approach is used when ultrasound shows patency of a significant length of a major hepatic vein, but this is inaccessible from the inferior vena cava (IVC).[59][60]

IVC stent insertion is usually needed when balloon dilation is insufficient alone, or when there is recoil, or recurrent stenosis.[63]

Transjugular intrahepatic portosystemic shunt (TIPS) is considered the most common interventional procedure for Budd-Chiari syndrome in Western countries.[66] For the shunt to function, the portacaval pressure gradient should be <10 mmHg.

Failure of angioplasty (because the remaining patent veins are too small or have insufficient flow) and the presence of diffuse hepatic vein thrombosis are indications for TIPS.

Elective TIPS may be performed in patients with refractory ascites, recurrent variceal bleeding, hepatic outflow obstruction resulting from compression of the intrahepatic IVC by a hypertrophied caudate lobe, or poor hepatic reserve precluding surgical shunting.

Consider – localised thrombolysis

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

localised thrombolysis

Additional treatment recommended for SOME patients in selected patient group

When a clot is fresh, thrombolytic therapy can be given alongside interventional treatment, by infusing a thrombolytic agent directly into the thrombosed vein.

Where there is venous obstruction of any cause and thrombus, localised thrombolytic therapy can be administered (via a catheter), prior to balloon dilatation or stenting of the obstructed vessel.[76]

Consider – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42]

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with Budd-Chiari syndrome. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – symptom control

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

symptom control

Additional treatment recommended for SOME patients in selected patient group

Ascites is treated with diuretics (e.g., furosemide, spironolactone). Combined diuretic therapy is recommended if response to single therapy is not satisfactory. If ascites is still not controlled by medical therapy, paracentesis may be required.

History of bleeding due to portal hypertension is managed with pharmacological or endoscopic therapy.[38]

Primary options

furosemide: 20-40 mg orally once daily

OR

spironolactone: 50-200 mg orally once daily

Secondary options

furosemide: 20-40 mg orally once daily

and

spironolactone: 50-200 mg orally once daily

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

3rd line – surgical shunting

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
3rd line – 

surgical shunting

Indicated in patients not improving by radiological angioplasty and if the portacaval venous pressure gradient is ≥10 mmHg.

Indicated if there is good hepatic reserve and insignificant fibrosis. Better surgical outcomes are seen in patients with Child-Pugh class A and where the underlying cause has a favourable long-term outcome, such as essential thrombocythaemia.[42]

Side-to-side portacaval shunts, side-to-side mesocaval shunts, and cavoatrial shunts are used for patients with both caval and hepatic venous obstruction.[71]

Mesoatrial shunt is indicated if the inferior vena cava is obstructed, thrombosed, or compressed by a hypertrophied caudate lobe, resulting in low portacaval pressure gradient (however, a transjugular intrahepatic portosystemic shunt is usually a better approach in this setting).

Inferior vena caval membranous obstruction is managed by transatrial membranectomy, or, less commonly, by dorsocranial resection of the liver with hepatico-atrial anastomosis.[71][72]

Consider – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42]

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with Budd-Chiari syndrome. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – symptom control

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

symptom control

Additional treatment recommended for SOME patients in selected patient group

Ascites is treated with diuretics (e.g., furosemide, spironolactone). Combined diuretic therapy is recommended if response to single therapy is not satisfactory. If ascites is still not controlled by medical therapy, paracentesis may be required.

History of bleeding due to portal hypertension is managed with pharmacological or endoscopic therapy.[38]

Primary options

furosemide: 20-40 mg orally once daily

OR

spironolactone: 50-200 mg orally once daily

Secondary options

furosemide: 20-40 mg orally once daily

and

spironolactone: 50-200 mg orally once daily

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

4th line – liver transplant

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
4th line – 

liver transplant

Indications for transplantation include advanced cirrhosis, and failure of non-surgical treatment approaches or portosystemic shunting.[38][73][74]

Seventy-five percent of patients with Budd-Chiari syndrome (BCS) have a detectable genetic hypercoagulable state. Liver transplantation can cure almost all hereditary thrombophilias; however, thrombosis can still occur and anticoagulation is necessary.[4]

Living-donor liver transplantation using modified cavoplasty is a highly effective treatment for BCS and, in conjunction with long-term anticoagulant therapy and interventional radiological treatment, provides good long-term survival.[75]

Consider – anticoagulation

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Most patients with Budd-Chiari syndrome (BCS) exhibit important risk factors for thrombosis; thus anticoagulation is best continued after transplantation.[38]

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation should be initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42] 

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with BCS. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

Consider – specific treatment of predisposing thrombophilia

Back
ACUTE
|
primary: non-fulminant
|
symptomatic: ≥72 hours from symptom onset
Consider – 

specific treatment of predisposing thrombophilia

Additional treatment recommended for SOME patients in selected patient group

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

primary: fulminant

1st line – emergency liver transplantation

Back
ACUTE
|
primary: fulminant
|
no delay in transplant availability
1st line – 

emergency liver transplantation

Emergency liver transplantation is indicated in patients with fulminant Budd-Chiari syndrome (BCS) and as a salvage procedure for fulminant hepatic failure resulting from surgical shunting.[73]

Living-donor liver transplantation using modified cavoplasty is a highly effective treatment for BCS and, in conjunction with long-term anticoagulation therapy and interventional radiological treatment, provides good long-term survival.[75]

Consider – anticoagulation

Back
ACUTE
|
primary: fulminant
|
no delay in transplant availability
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Most patients with Budd-Chiari syndrome (BCS) exhibit important risk factors for thrombosis; thus anticoagulation is best continued after transplantation.[38]

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42]

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with BCS. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

More

1st line – bridging transjugular intrahepatic portosystemic shunts (TIPS)

Back
ACUTE
|
primary: fulminant
|
delay in transplant availability
1st line – 

bridging transjugular intrahepatic portosystemic shunts (TIPS)

TIPS is done on an emergency basis in fulminant Budd-Chiari syndrome (BCS) to serve as a bridge to liver transplantation.[1][2]​​​

TIPS is considered the most common interventional procedure for BCS in Western countries.[66] For the shunt to function, the portacaval pressure gradient should be <10 mmHg.

Consider – anticoagulation

Back
ACUTE
|
primary: fulminant
|
delay in transplant availability
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Most patients with Budd-Chiari syndrome (BCS) exhibit important risk factors for thrombosis; thus anticoagulation is best continued after transplantation.[38]

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (worsening symptoms, poorly controlled ascites, rising serum transaminases).[42]

Use low molecular weight heparin (e.g., enoxaparin, dalteparin) as unfractionated heparin is generally not recommended for the management of patients with BCS. Patients should be given long-term anticoagulation.[2]​ Follow with a vitamin K antagonist (e.g., warfarin) unless there is active bleeding or a very high bleeding risk.

Primary options

enoxaparin: 40 mg subcutaneously once daily

or

dalteparin: 5000 units subcutaneously once daily

-- AND --

warfarin: 2-10 mg orally once daily initially, adjust dose according to target INR

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Plus – liver transplantation

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ACUTE
|
primary: fulminant
|
delay in transplant availability
Plus – 

liver transplantation

Treatment recommended for ALL patients in selected patient group

Emergency liver transplantation is indicated in patients with fulminant Budd-Chiari syndrome (BCS) and as a salvage procedure for fulminant hepatic failure resulting from surgical shunting.[73]

Living-donor liver transplantation using modified cavoplasty is a highly effective treatment for BCS and, in conjunction with long-term anticoagulation therapy and interventional radiological treatment, provides good long-term survival.[75]

secondary Budd-Chiari syndrome

1st line – surgical removal of extrahepatic obstructing lesion

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ACUTE
|
secondary Budd-Chiari syndrome
1st line – 

surgical removal of extrahepatic obstructing lesion

Surgical removal of the compressing cyst, abscess, or invading tumour is required.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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