The utility of TIPS in the management of Budd-Chiari syndrome.
OBJECTIVE
Budd-Chiari syndrome (BCS) is a rare condition associated with hepatic venous outflow obstruction classically treated with portosystemic shunts or liver transplantation. Recent reports indicate promising results with the use of transjugular intrahepatic portosystemic shunts (TIPS) in the treatment of these patients.


METHODS
We reviewed a 10-year single-institution experience with TIPS in patients diagnosed with BCS.


RESULTS
Eleven patients with BCS underwent TIPS procedures, 3 of whom carried a diagnosis of paroxysmal nocturnal hemoglobinuria, a relative contraindication for liver transplantation. One TIPS procedure was unsuccessful for technical reasons. No patient suffered mortality or major morbidity related to the TIPS procedure. The mean reduction of portal venous pressures was 43.7%, with a mean decrease of 73% in the pressure gradient. Of the 7 patients where long-term follow-up was available, 57% had shunts which remained patent but required several nonsurgical revisions for occlusion, with an average assisted patency of 37.5 months.


CONCLUSIONS
TIPS is an effective modality in the treatment of patients with BCS, especially for those who are not candidates for liver transplantation. TIPS can be successfully used as a bridge to surgical portosystemic shunting, as well as liver transplantation, but may cause technical difficulties when performing transplantation.
Molmenti
Ernesto P
EP
Department of Surgery, Division of Transplantation, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Segev
Dorry L
DL
Arepally
Aravind
A
Hong
Jenny
J
Thuluvath
Paul J
PJ
Rai
Rudra
R
Klein
Andrew S
AS
D006502:Budd-Chiari Syndrome / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D011169:Portal Vein / Q000503:physiopathology; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D012189:Retrospective Studies