Management of portal hypertension, Budd–Chiari syndrome and portal vein thrombosis

Portal hypertension is associated with many of the known complications of cirrhosis and has an enormous impact on a patient's prognosis. Ascites and hepatic encephalopathy represent the most common complications of cirrhosis; both are associated with a significantly worse prognosis, with 50% survival over the next 1–2 years. Acute variceal bleeding is a life-threatening complication and represents a leading cause of death in patients with cirrhosis. With advances in care, such as prophylactic antibiotics, vasoactive drugs and early transjugular intrahepatic portosystemic shunt (TIPSS) in patients with bleeding refractory to early endoscopic management, the mortality rate has significantly improved but remains 15–20%. Secondary prophylaxis of variceal bleeding with non-selective β-adrenoceptor blockers and/or endoscopic variceal ligation has also improved survival. Budd–Chiari syndrome (BCS) is a life-threatening disorder resulting from hepatic venous outflow obstruction. Myeloproliferative neoplasms (MPN) represent the most common cause of BCS, although a significant proportion of patients have more than one risk factor. Therapeutic anticoagulation remains the first-line treatment for both BCS and symptomatic portal vein thrombosis. TIPSS is increasingly used in the management of BCS and can reduce the need for liver transplantation.