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Acquired von Willebrand syndrome in aortic stenosis.
N Engl J Med 2003; 349(4):343-9NEJM

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+Show Affiliations

Equipe d'Accueil 2693, University of Lille II, Faculté de Médecine, Lille, France.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

12878741

Citation

Vincentelli, André, et al. "Acquired Von Willebrand Syndrome in Aortic Stenosis." The New England Journal of Medicine, vol. 349, no. 4, 2003, pp. 343-9.
Vincentelli A, Susen S, Le Tourneau T, et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med. 2003;349(4):343-9.
Vincentelli, A., Susen, S., Le Tourneau, T., Six, I., Fabre, O., Juthier, F., ... Jude, B. (2003). Acquired von Willebrand syndrome in aortic stenosis. The New England Journal of Medicine, 349(4), pp. 343-9.
Vincentelli A, et al. Acquired Von Willebrand Syndrome in Aortic Stenosis. N Engl J Med. 2003 Jul 24;349(4):343-9. PubMed PMID: 12878741.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand syndrome in aortic stenosis. AU - Vincentelli,André, AU - Susen,Sophie, AU - Le Tourneau,Thierry, AU - Six,Isabelle, AU - Fabre,Olivier, AU - Juthier,Francis, AU - Bauters,Anne, AU - Decoene,Christophe, AU - Goudemand,Jenny, AU - Prat,Alain, AU - Jude,Brigitte, PY - 2003/7/25/pubmed PY - 2003/7/29/medline PY - 2003/7/25/entrez SP - 343 EP - 9 JF - The New England journal of medicine JO - N. Engl. J. Med. VL - 349 IS - 4 N2 - 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. SN - 1533-4406 UR - https://www.unboundmedicine.com/medline/citation/12878741/Acquired_von_Willebrand_syndrome_in_aortic_stenosis_ L2 - http://www.nejm.org/doi/full/10.1056/NEJMoa022831?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -