The transjugular intrahepatic portosystemic shunt (TIPS) for budd-chiari syndrome

Abstract

Since its introduction in 1988, the transjugular intrahepatic portosystemic shunt (TIPS) has become an important treatment for the Budd-Chiari syndrome (BCS). It almost completely replaced surgical shunt treatment and reduced the need of liver transplantation to few cases where TIPS treatment failed. The TIPS intervention is always on top of the medical treatment which consists of anticoagulation and specific treatment of an underlying hematological disease. With the advent of transcaval puncture in cases with occluded hepatic veins, TIPS can successfully be implanted in more than 95% of patients with a very low complication rate. With the use of PTFE-covered stents, long-term patency of the shunt is acceptable with revision required in 40% of patients during 2 years of follow- up and a secondary patency rate of almost 100%. The present clinical practice guidelines of the European Association for the Study of the Liver recommend a stepwise therapeutic algorithm by order of increasing invasiveness beginning with anticoagulation, angioplasty in patients with web-like BCS, TIPS, and finally, liver transplantation. However, medical treatment is ineffective in more than 80-90% of patients. In addition, it may prolong the time of insufficient hepatic blood supply which may result in disease progression. In contrast, the TIPS leads to a rapid and effective drainage of the hepatic and splanchnic vascular beds, thus improving hepatic function and ameliorating portal hypertension. In this regard, early vascular intervention, e.g., TIPS or angioplasty in cases of short BCS, may be favorable in patients with acute and subacute disease. Survival after TIPS is excellent in both, acute and chronic BCS with a 5 and 10-year survival rate of 90% and 80%, respectively. About half of the patients die from their underlying hematological disease. The TIPS compares favorably with surgical shunt treatment and primary liver transplantation. In addition, it does not compromise later liver transplantation, a fact which justifies a strategy which places liver transplantation at the very end.