title: Acquired von Willebrand syndrome in aortic stenosis.
abstract: BACKGROUND
  Aortic-valve stenosis can be complicated by bleeding that is associated with acquired type 2A von Willebrand syndrome. However, the prevalence and cause of the hemostatic abnormality in aortic stenosis are unknown.
  
  
  METHODS
  We enrolled 50 consecutive patients with aortic stenosis, who completed a standardized screening questionnaire to detect a history of bleeding. Forty-two patients with severe aortic stenosis underwent valve replacement. Platelet function under conditions of high shear stress, von Willebrand factor collagen-binding activity and antigen levels, and the multimeric structure of von Willebrand factor were assessed at base line and one day, seven days, and six months postoperatively.
  
  
  RESULTS
  Skin or mucosal bleeding occurred in 21 percent of the patients with severe aortic stenosis. Platelet-function abnormalities under conditions of high shear stress, decreased von Willebrand factor collagen-binding activity and the loss of the largest multimers, or a combination of these was present in 67 to 92 percent of patients with severe aortic stenosis and correlated significantly with the severity of valve stenosis. Primary hemostatic abnormalities were completely corrected on the first day after surgery but tended to recur at six months, especially when there was a mismatch between patient and prosthesis (with an effective orifice area of less than 0.8 cm2 per square meter of body-surface area).
  
  
  CONCLUSIONS
  Type 2A von Willebrand syndrome is common in patients with severe aortic stenosis. Von Willebrand factor abnormalities are directly related to the severity of aortic stenosis and are improved by valve replacement in the absence of mismatch between patient and prosthesis.
authors.affiliation: Equipe d'Accueil 2693, University of Lille II, Faculté de Médecine, Lille, France.
authors.forename: André
authors.initials: A
authors.lastname: Vincentelli
authors.forename: Sophie
authors.initials: S
authors.lastname: Susen
authors.forename: Thierry
authors.initials: T
authors.lastname: Le Tourneau
authors.forename: Isabelle
authors.initials: I
authors.lastname: Six
authors.forename: Olivier
authors.initials: O
authors.lastname: Fabre
authors.forename: Francis
authors.initials: F
authors.lastname: Juthier
authors.forename: Anne
authors.initials: A
authors.lastname: Bauters
authors.forename: Christophe
authors.initials: C
authors.lastname: Decoene
authors.forename: Jenny
authors.initials: J
authors.lastname: Goudemand
authors.forename: Alain
authors.initials: A
authors.lastname: Prat
authors.forename: Brigitte
authors.initials: B
authors.lastname: Jude
mesh_terms: D000368:Aged; D001024:Aortic Valve Stenosis / Q000097:blood / Q000150:complications* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation; D018056:Hemorheology; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D019106:Postoperative Hemorrhage; D015995:Prevalence; D011795:Surveys and Questionnaires; D014842:von Willebrand Diseases / Q000209:etiology*; D014841:von Willebrand Factor / Q000737:chemistry / Q000378:metabolism*
chemical_list: D014841:von Willebrand Factor