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 patients’ prognosis. Ascites and hepatic encephalopathy represent the most common complications of cirrhosis; both are associated with a significantly worse prognosis, with 50% survival over 1–2 years. Acute variceal bleeding is a life-threatening complication, and despite significant improvements in the management it remains a leading cause of death in patients with cirrhosis. Advances in variceal bleeding management, including empirical antibiotic use, vasoactive drugs, early endoscopy and therapies such as transjugular intrahepatic portosystemic shunt (TIPSS) in patients with refractory bleeding, have resulted in improved mortality rates, currently around 11–20% per episode. Secondary prophylaxis of variceal bleeding with a combination of non-selective β-adrenoceptor blockers and endoscopic variceal ligation has also improved survival. Budd–Chiari syndrome (BCS) is a life-threatening disorder resulting from hepatic venous outflow obstruction. Myeloproliferative neoplasms 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.