Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident. Recent data support that in BCS liver fibrosis could arise from chronic microvascular ischemia. A reasoning model of BCS physiopathology is that impaired hepatic vein outflow has hemodynamic consequences on portal hypertension development and causes hepatic fibrosis and liver failure through chronic ischemic damage. On this assumption is the concept that relieving liver congestion could ameliorate liver function and prevent development of BCS complications. Recently, early interventional treatment with TIPS for BCS has been reported to be effective. Early TIPS seems to be the best option for BCS management. Future multicenter controlled studies should compare the outcome of BCS treated with early interventional treatment compared with stepwise strategy.
Keywords: Budd-Chiari Syndrome; liver transplant; outcome; transjugular intrahepatic portosystemic shunt (TIPS).