title: Liver transplantation for hereditary hemorrhagic telangiectasia: Report of the European liver transplant registry.
abstract: BACKGROUND
  Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a rare disease characterized by the presence of arteriovenous malformations. Hepatic involvement can lead to life-threatening conditions.
  
  
  METHODS
  Forty patients, reported to the European Liver Transplant Registry, were analyzed to define the role of liver transplantation in the treatment of the hepatic disease form. Indications for transplantation were classified according to Garcia-Tsao: cardiac failure (14 patients), biliary necrosis causing hepatic failure (12 patients), severe portal hypertension (5 patients), cardiac failure and biliary necrosis (6 patients), cardiac failure and portal hypertension (2 patients), and cardiac failure associated with biliary necrosis and portal hypertension (1 patient). Eighteen (81%) of 22 patients had pulmonary artery hypertension. Twelve (30%) patients had pretransplant hepatic interventions. Follow-up was complete for all patients with a mean of 69 months (range, 0-230 months).
  
  
  RESULTS
  One-, 5- and 10-year actuarial patient and graft survival rates are 82.5%. Six of the 7 pretransplant procedures performed on the hepatic artery were severely complicated. Cardiovascular function documented in 24 patients improved in 18 patients and remained stable in 5 patients; 1 patient died perioperatively of acute heart failure. Twenty-four (60%) patients had post-transplant complications, all but one occurring within the first 4 posttransplant months. Seven (17.5%) patients died perioperatively, 6 of them due to bleeding and 1 due to cardiac failure; 1 (2.5%) patient died late due to chronic rejection. There were 2 possible recurrences. Quality of life markedly improved in all 32 surviving patients.
  
  
  CONCLUSIONS
  The results of the largest reported transplant series in the treatment of hepatic-based HHT are excellent. Elimination of hepatobiliary sepsis and reversal of cardiopulmonary changes dramatically improve quality of life of the recipients. LT should be proposed earlier in the course of symptomatic hepatic HHT presenting with life-threatening conditions. Palliative interventions, especially on the hepatic artery, should be avoided in view of their high (infectious) complication rate.
authors.affiliation: Liver Transplant Unit, University Hospital St. Luc, Brussels, Belgium. lerut@chir.ucl.ac.be
authors.forename: Jan
authors.initials: J
authors.lastname: Lerut
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Orlando
authors.forename: René
authors.initials: R
authors.lastname: Adam
authors.forename: Carlo
authors.initials: C
authors.lastname: Sabbà
authors.forename: Robert
authors.initials: R
authors.lastname: Pfitzmann
authors.forename: Jurgen
authors.initials: J
authors.lastname: Klempnauer
authors.forename: Jacques
authors.initials: J
authors.lastname: Belghiti
authors.forename: Jacques
authors.initials: J
authors.lastname: Pirenne
authors.forename: Thierry
authors.initials: T
authors.lastname: Thevenot
authors.forename: Christian
authors.initials: C
authors.lastname: Hillert
authors.forename: Colin M
authors.initials: CM
authors.lastname: Brown
authors.forename: Dominique
authors.initials: D
authors.lastname: Gonze
authors.forename: Vincent
authors.initials: V
authors.lastname: Karam
authors.forename: Olivier
authors.initials: O
authors.lastname: Boillot
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005060:Europe; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012042:Registries; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000473:pathology / Q000601:surgery*; D016896:Treatment Outcome