Timing of transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: An Italian hepatologist’s perspective

Abstract

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.

Budd–Chiari Syndrome Management: Controversies and Open Issues

“…Usually, one or more prothrombotic conditions, such as myeloproliferative disorders (MPD), are found. However, a number of cases remain idiopathic [ 1 , 2 , 3 , 4 , 5 ]. Furthermore, prothrombotic disorders are more frequent in the West than in Asia, where HVs involvement is more common and BCS is generally due to IVC involvement.…”

“…It seems realistic to speculate that BCS in the West and East is the same syndrome within different diseases [ 7 ]. Recently, however, new perspectives of BCS have begun to emerge [ 1 ].…”

“…The main complications of BCS are portal hypertension and development of HCC, though the latter is rare [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ]. Furthermore, in about 10% of cases, underlying disease could negatively evolve, just like MPD, thus affecting BCS prognosis [ 23 , 24 ].…”

“…Overall, previous classifications and prognostic indexes of BCS are of limited value, particularly following the widespread implementation of interventional treatments [ 1 , 2 , 3 , 4 , 5 , 6 ]. A recently proposed pragmatic clinical classification identifies two clinical phases [ 23 ]: an asymptomatic (or pauci-symptomatic) phase (AP) or clinically silent thrombosis, and a symptomatic phase (SP).…”

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

“…Recent data challenge current guidelines that recommend TIPS after failure of anticoagulation therapy, as early TIPS seems to reduce the long-term effects of microvascular ischemia which eventually lead to liver failure. Several authors suggest early TIPS in adjunct to anticoagulation, as the safety and effectiveness of the procedure justifies a more aggressive approach in order to reduce the development of hepatic fibrosis and liver failure, caused by chronic venous conjunction and portal hypertension[ 87 ]. Other authors propose decompressive procedures including early TIPS only in patients with signs of portal hypertension and recommend that medical therapy as sole treatment should be reserved only for patients without any signs of portal hypertension[ 9 ].…”