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Budd-Chiari syndrome

Definition
BCS is a liver disease that results from hepatic venous outflow obstruction characterized by a triad of abdominal discomfort, hepatomegaly, and ascites.
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Pathophysiology
Etiologies of BCS can be broadly classified into thrombotic causes (primary myeloproliferative diseases, underlying thrombophilia) and non-thrombotic causes (compression due to tumors of adjacent organs, polycystic kidney disease, parasitic liver disease).
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Disease course
Obstruction of hepatic veins increases sinusoidal pressure and reduces sinusoidal blood blow, leading to clinical manifestations of acute abdominal pain, ascites, and hepatomegaly, or chronic symptoms of long-standing portal hypertension. Disease progression may result in fibrosis and fulminant hepatic failure.
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Prognosis and risk of recurrence
1-year and 5-year mortality rate of treated BCS is 6% and 13%, respectively.
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Key sources
The following summarized guidelines for the evaluation and management of Budd-Chiari syndrome are prepared by our editorial team based on guidelines from the American Society of Hematology (ASH 2023), the European Association for the Study of the Liver (EASL 2023; 2016), the American Association for the Study of Liver Diseases (AASLD 2021), the American College of Gastroenterology (ACG 2020), the Italian Association for the Study of the Liver (AISF 2016), and the Baveno VI Consensus Workshop (Baveno VI 2015).
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Guidelines

1.Screening and diagnosis

Diagnosis: as per AASLD 2021 guidelines, suspect the diagnosis of BCS in any patient with liver disease (recent or chronic) of unknown etiology, especially with a higher suspicion in a patient with a recognized prothrombotic disorder.
E
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2.Diagnostic investigations

Diagnostic imaging
As per AASLD 2021 guidelines:
Obtain Doppler ultrasound, performed by an experienced operator, as the first-line imaging modality for BCS.
E
Consider obtaining CT or MRI for diagnostic confirmation and interventional planning.
E

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  • Prothrombotic workup

3.Medical management

General principles: as per AASLD 2021 guidelines, offer a progressive step-up therapeutic strategy according to the clinical response from less to more invasive therapies in patients with BCS.
E

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  • Anticoagulant therapy (initiation)

  • Anticoagulant therapy (duration)

4.Therapeutic procedures

Hepatic vein or inferior vena cava angioplasty: as per ACG 2020 guidelines, perform angioplasty in patients with BCS if medical therapy fails, as determined by worsening liver and/or renal function, ascites, or hepatic encephalopathy.
B

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  • TIPS

5.Surgical interventions

Surgical portosystemic shunt
As per AASLD 2021 guidelines:
Reserve liver transplantation for patients with BCS when medical and vascular interventional approaches fail.
E
Consider performing liver transplantation as the first step in patients with ALF. Consider performing TIPS/direct intrahepatic portocaval shunt in patients with ALF while waiting for transplant because it may improve liver function and potentially avoid the need for transplantation.
E

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  • Liver transplantation

6.Specific circumstances

Pregnant patients: as per EASL 2023 guidelines, counsel female patients with vascular liver disease that the condition is associated with preterm birth and operative delivery.
B

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  • Patients with portal hypertension

7.Follow-up and surveillance

Indications for referral: as per AASLD 2021 guidelines, refer patients with BCS to tertiary care centers with expertise in this disorder.
E

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  • Surveillance for HCC