Budd-Chiari syndrome Diagnosis and management Diagnosis EASL Q Does the patient have clinical, biochemical or radiological evidence of acute or chronic liver disease? No Consider alternative diagnoses. Yes Assess the probability of BCS in any symptomatic or asymptomatic patient with acute or chronic liver disease. A Diagnostic imaging Obtain Doppler ultrasound as the first line of investigation for BCS, and use MRI or CT for diagnostic confirmation. A Prothrombotic workup EASL Q Do radiologic investigations confirm the diagnosis of Budd-Chiari syndrome? No Consider alternative diagnoses. Yes Evaluate patients with BCS for underlying local and systemic prothrombotic factors, including intra-abdominal inflammatory conditions and abdominal malignancies, inherited and acquired thrombophilias, myeloproliferative neoplasms, paroxysmal nocturnal Hgburia and autoimmune disorders. Do not stop evaluating for additional risk factors if one risk factor is identified. A Obtain protein S, protein C and antithrombin levels, FVL mutation, prothrombin G20210A gene variant and antiphospholipid antibodies for thrombophilia screening in patients with BCS. Obtain repeated antiphospholipid antibodies testing after 12 weeks, if initially positive. A Obtain JAK2V617F mutation testing for evaluation for myeloproliferative neoplasms in patients with BCS, and in individuals with normal peripheral blood cell counts. A Obtain CALR mutation testing in JAK2V617F mutation negative patients, and consider performing bone marrow histology if both are negative. Refer these patients to a hematologist. B Anticoagulant therapy, initiation Initiate anticoagulant therapy in patients with BCS in the absence of major contraindications. A Recognize that portal hypertension complications, when adequately treated, are not a contraindication for anticoagulation. B General principles Treat any underlying prothrombotic condition appropriately. B Hepatic vein or inferior vena cava angioplasty EASL Q Does the patient show an adequate response to medical treatment? Yes Continue management and follow-up as clinically appropriate. No Perform angioplasty/stenting as the first-line decompressive procedure in patients with short hepatic vein stenosis or IVC stenosis. A Transjugular intrahepatic portosystemic shunt EASL Q Does the patient show an adequate response to medical treatment with angioplasty/stenting? Yes Continue management and follow-up as clinically appropriate. No Perform a portal derivative technique in patients not responding to initial therapy or angioplasty/stenting. A Perform TIPS implantation, unless contraindicated, as the first-line portal derivative technique. A Surgical portosystemic shunt EASL Q Is TIPS contraindicated? Q Does TIPS fail to achieve an adequate response? Yes to any Continue management and follow-up as clinically appropriate. No to all Discuss surgical shunting when TIPS is not feasible or fails for patients not responding to initial therapy, or not responding to angioplasty/stenting with portal derivative techniques. B Liver transplantation EASL Q Does the patient show an adequate response to surgical portal derivation? Yes Continue management and follow-up as clinically appropriate. No Propose liver transplantation as a salvage treatment for appropriate candidates in whom derivative techniques have failed. A