title: Etiology, management, and outcome of the Budd-Chiari syndrome.
abstract: BACKGROUND
  The Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction. What is known about the syndrome is based on small studies of prevalent cases.
  
  
  OBJECTIVE
  To characterize the causes and treatment of incident BCS.
  
  
  METHODS
  Consecutive case series of patients with incident BCS, enrolled from October 2003 to October 2005 and followed until May 2006.
  
  
  METHODS
  Academic and nonacademic hospitals in France, Spain, Italy, Great Britain, Germany, Belgium, the Netherlands, Portugal, and Switzerland.
  
  
  METHODS
  Persons older than 16 years with definite hepatic outflow obstruction diagnosed by imaging. Persons with hepatic outflow obstruction due to heart failure, sinusoidal obstruction syndrome, cancer, or liver transplantation were excluded.
  
  
  METHODS
  Signs and symptoms; laboratory and imaging findings; diagnosis; treatment; and overall, transplantation-free, and intervention-free survival.
  
  
  RESULTS
  163 incident cases of BCS were identified. Median follow-up was 17 months (range, 0.1 to 31 months). Most patients (84%) had at least 1 thrombotic risk factor, and many (46%) had more than 1; the most common was myeloproliferative disorders (49% of 103 tested patients). Patients were mainly treated with anticoagulation (140 patients [86%]), transjugular intrahepatic portosystemic shunting (56 patients [34%]), or liver transplantation (20 patients [12%]), and 80 patients (49%) were managed noninvasively. Only 3 patients underwent surgical shunting. The survival rate was 87% (95% CI, 82% to 93%) at 1 year and 82% (CI, 75% to 88%) at 2 years.
  
  
  CONCLUSIONS
  Treatment was not standardized across all centers, and data on important clinical variables were missing for some patients.
  
  
  CONCLUSIONS
  Most patients with BCS have at least 1 thrombotic risk factor, and many have more than 1; myeloproliferative disorders are most common. One- and 2-year survival rates are good with contemporary management, which includes noninvasive therapies (anticoagulation and diuretics) and invasive techniques. Transjugular intrahepatic portosystemic shunting seems to have replaced surgical shunting as the most common invasive therapeutic procedure.
  
  
  BACKGROUND
  Fifth Framework Programme of the European Commission.
authors: Erasmus MC University Hospital Rotterdam, Rotterdam, the Netherlands.
authors: Sarwa
authors: S
authors: Darwish Murad
authors: Aurelie
authors: A
authors: Plessier
authors: Manuel
authors: M
authors: Hernandez-Guerra
authors: Federica
authors: F
authors: Fabris
authors: Chundamannil E
authors: CE
authors: Eapen
authors: Matthias J
authors: MJ
authors: Bahr
authors: Jonel
authors: J
authors: Trebicka
authors: Isabelle
authors: I
authors: Morard
authors: Luc
authors: L
authors: Lasser
authors: Joerg
authors: J
authors: Heller
authors: Antoine
authors: A
authors: Hadengue
authors: Philippe
authors: P
authors: Langlet
authors: Helena
authors: H
authors: Miranda
authors: Massimo
authors: M
authors: Primignani
authors: Elwyn
authors: E
authors: Elias
authors: Frank W
authors: FW
authors: Leebeek
authors: Frits R
authors: FR
authors: Rosendaal
authors: Juan-Carlos
authors: JC
authors: Garcia-Pagan
authors: Dominique C
authors: DC
authors: Valla
authors: Harry L A
authors: HL
authors: Janssen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015906:Angioplasty, Balloon, Coronary; D006502:Budd-Chiari Syndrome / Q000209:etiology* / Q000401:mortality / Q000628:therapy*; D005060:Europe; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000379:methods; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D011446:Prospective Studies; D012307:Risk Factors; D019851:Thrombophilia / Q000150:complications; D016896:Treatment Outcome; D055815:Young Adult