title: TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy.
abstract: Patients with Budd-Chiari syndrome (BCS) may require treatment with portal decompressive surgery or liver transplantation. Transjugular intrahepatic portosystemic shunt (TIPS) represents a new treatment alternative, but its long-term effect on BCS outcome has not been evaluated. Twenty-one patients with BCS consecutively admitted to our unit were evaluated. The mean follow-up was 4 +/- 3 years. Seven patients had nonprogressive forms and were successfully controlled with medical therapy; 1 case, with a short-length hepatic vein stenosis was successfully treated by angioplasty. All 8 patients are alive and asymptomatic. The remaining 13 patients, had a TIPS because of clinical deterioration (in one of them, because early TIPS thrombosis a successful side-to-side portacaval shunt [SSPCS] was performed) followed by an improvement in clinical condition. However, a patient with fulminant liver failure before TIPS insertion, died 4 months later and another patient with cirrhosis at diagnosis had liver transplantation 2 years later. The remaining 11 patients are alive and free of ascites. In 3 of these patients TIPS is patent after 3, 6, and 12 months. The remaining 8 patients developed late TIPS dysfunction. In two of these cases, after angioplasty and restenting, TIPS is patent after a follow-up of 9 and 80 months. In 5 other patients, recurring TIPS occlusion was not further corrected because no signs of portal hypertension were present. In conclusion, in patients with BCS uncontrolled with medical therapy, TIPS is a highly effective technique that is associated with long-term survival.
authors.affiliation: Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, University of Barcelona, Barcelona, Catalunya, Spain.
authors.forename: Antonia
authors.initials: A
authors.lastname: Perelló
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: García-Pagán
authors.forename: Rosa
authors.initials: R
authors.lastname: Gilabert
authors.forename: Yanette
authors.initials: Y
authors.lastname: Suárez
authors.forename: Eduardo
authors.initials: E
authors.lastname: Moitinho
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Reverter
authors.forename: Angels
authors.initials: A
authors.lastname: Escorsell
authors.forename: Jaume
authors.initials: J
authors.lastname: Bosch
authors.forename: Juan
authors.initials: J
authors.lastname: Rodés
mesh_terms: D015746:Abdominal Pain; D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D005260:Female; D006503:Hepatic Veins / Q000473:pathology / Q000601:surgery; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D015996:Survival Rate; D016896:Treatment Outcome; D014463:Ultrasonography; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D005723:gamma-Glutamyltransferase