title: [Liver transplantation for intrahepatic Rendu-Osler-Weber's disease: the Paul Brousse hospital experience].
abstract: Nine centers have reported 15 cases of liver transplantation for Rendu-Osler-Weber's disease with liver involvement. Six cases are reported to analyse the specific technical and hemodynamical aspects.
  
  
  METHODS
  Five women and 1 man were transplanted for Rendu-Osler-Weber's disease. The clinical presentation was biliary disease in 3 cases, portal hypertension in 2 cases, cardiac failure in 1 case. Systemic hemodynamics were assessed at the beginning and at the end of the transplantation procedure.
  
  
  RESULTS
  The procedure lasted from 11 to 15 hours (median=13 hours and 15 minutes). Blood transfusion during the procedure varied from 16 to 88 blood units (median=59 blood units). Six patients had hyperkinetic syndrome at the beginning of the procedure. At the end of transplantation, mean arterial pressure significantly increased (from 66 +/- 2 to 72 +/- 6 mmHg, p<0.05), whereas cardiac output (from 9.2 +/- 3.0 à 5.7 +/- 0.5 L/mn, p<0.05) significantly decreased. Two patients died at D2 and D11 and 4 are alive 3 to 7.5 years (median=4 years 9 months) after transplantation with a normal liver function and without any cardiac symptoms.
  
  
  CONCLUSIONS
  Liver transplantation for Rendu-Osler-Weber's disease is a difficult procedure. When successful, liver transplantation is curative of both the liver disease and the hyperkinetic state.
authors.affiliation: Département de Chirurgie Hépato-Biliaire, Hôpital Paul Brousse, Université Paris-Sud, UPRES 1596, IFR 89.9, Villejuif, France. daniel.azoulay@pbr.ap-hop-paris.fr
authors.forename: Daniel
authors.initials: D
authors.lastname: Azoulay
authors.forename: Sophie
authors.initials: S
authors.lastname: Precetti
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Emile
authors.forename: Philippe
authors.initials: P
authors.lastname: Ichai
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Gillon
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Duclos-Vallée
authors.forename: Selim
authors.initials: S
authors.lastname: Visda
authors.forename: René
authors.initials: R
authors.lastname: Adam
authors.forename: Denis
authors.initials: D
authors.lastname: Castaing
authors.forename: Didier
authors.initials: D
authors.lastname: Samuel
authors.forename: Henri
authors.initials: H
authors.lastname: Bismuth
mesh_terms: D000328:Adult; D000368:Aged; D002761:Cholangitis / Q000209:etiology / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D005602:France; D006333:Heart Failure / Q000209:etiology / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000601:surgery*; D016896:Treatment Outcome