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 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 remains a life-threatening complication and leading cause of death in individuals with cirrhosis. Advances in variceal bleeding management, including empirical antibiotic use, vasoactive drugs, early endoscopy and therapies such as transjugular intrahepatic portosystemic shunts (TIPS), have resulted in improved mortality rates, currently 11–20% per episode. The use of non-selective β-adrenoceptor blockers (NSBBs) in patients with clinically significant portal hypertension lowers the risk of variceal bleeding and improves patient outcomes; carvedilol is the preferred agent. Secondary prophylaxis of variceal bleeding with a combination of NSBBs and endoscopic variceal ligation also improves survival. Budd–Chiari syndrome (BCS) is a life-threatening disorder resulting from hepatic venous outflow obstruction. Myeloproliferative neoplasms represent its most common cause, although a significant proportion of patients have >1 risk factor. Therapeutic anticoagulation remains the first-line treatment for both BCS and symptomatic portal vein thrombosis. TIPS is increasingly used in the management of BCS and reduces the need for liver transplantation.