Premium
Treatment of Budd‐Chiari syndrome in a Liver Transplant Unit, the role of transjugular intrahepatic porto‐systemic shunt and liver transplantation
Author(s) -
Attwell A.,
Ludkowski M.,
Nash R.,
Kugelmas M.
Publication year - 2004
Publication title -
alimentary pharmacology and therapeutics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.308
H-Index - 177
eISSN - 1365-2036
pISSN - 0269-2813
DOI - 10.1111/j.1365-2036.2004.02190.x
Subject(s) - medicine , liver transplantation , budd–chiari syndrome , surgery , transplantation , portal hypertension , ascites , transjugular intrahepatic portosystemic shunt , shunt (medical) , cirrhosis , liver disease , fulminant , gastroenterology , inferior vena cava
Summary Background : Budd‐Chiari syndrome is an uncommon cause of liver failure usually associated with an underlying hypercoagulable state. Aim : To evaluate current trends in management of Budd‐Chiari syndrome at our institution. Methods : Twenty‐two patients with Budd‐Chiari syndrome underwent transjugular intrahepatic porto‐systemic shunt, liver transplantation, or both in between 1992 and 2001. We analysed underlying diagnosis, medical therapy, complications, follow‐up and overall outcomes. Results: Five patients (17%) presented with fulminant liver failure and 17 patients (83%) with new‐onset ascites or chronic liver disease. Seventeen patients (74%) underwent transjugular intrahepatic porto‐systemic shunt: improvement or stabilization occurred initially in 14 (82%), whereas the other three patients died within a month. At a mean 3 years follow‐up eight patients (47%) continued to do well clinically and four have died (23.5%); seven have required transjugular intrahepatic porto‐systemic shunt revisions (mean 2.3 interventions), five have experienced transjugular intrahepatic porto‐systemic shunt occlusion managed with new transjugular intrahepatic porto‐systemic shunt placement and five patients underwent subsequent transplantation. Of the 10 patients who underwent liver transplantation, patient and graft survival are 80% at a mean 5.7 years of follow‐up. No patient developed post‐transplant Budd‐Chiari syndrome. Conclusions : Transjugular intrahepatic porto‐systemic shunt is usually feasible in patients with Budd‐Chiari syndrome, and is best suited as a bridge to more timely liver transplantation. Long‐term success of transjugular intrahepatic porto‐systemic shunt is limited and usually requires revision, placement of a new shunt or liver transplantation. Liver transplantation with chronic anticoagulation offers excellent short‐ and medium‐term patient and graft survival. In our series, there was no recurrence of Budd‐Chiari syndrome after liver transplantation.