Acquired von Willebrand Syndrome in Aortic Stenosis

André Vincentelli, M.D., Sophie Susen, M.D., Thierry Le Tourneau, M.D., Ph.D., Isabelle Six, Ph.D., Olivier Fabre, M.D., Francis Juthier, Anne Bauters, Christophe Decoene, M.D., Jenny Goudemand, M.D., Ph.D., Alain Prat, M.D., and Brigitte Jude, M.D., Ph.D.


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.



Source Information

From the Equipe d'Accueil 2693, University of Lille II, Faculté de Médecine (A.V., S.S., T.L., O.F., F.J., A.B., J.G., A.P., B.J.), the Clinique de Chirurgie Cardiovasculaire (A.V., O.F., F.J., C.D., A.P.), the Institut d'Hématologie Biologique et d'Hémobiologie–Transfusion (S.S., I.S., A.B., J.G., B.J.), and the Service d'Explorations Fonctionnelles Cardiologiques (T.L.) — all in Lille, France.

Drs. Vincentelli and Susen contributed equally to this article.

Address reprint requests to Dr. Jude at the Institut d'Hématologie Biologique et d'Hémobiologie–Transfusion, Hôpital Cardiologique, 59037 Lille CEDEX, France.

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