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Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation.
J Thromb Haemost 2014; 12(12):1966-74JT

Abstract

BACKGROUND

Mitral valve regurgitation is associated with an acquired hemostatic defect.

OBJECTIVE

We sought to assess the prevalence and severity of acquired von Willebrand syndrome in patients with native valve mitral regurgitation (MR).

PATIENTS/METHODS

Fifty-three patients were prospectively observed with bleeding questionnaires and laboratory tests when undergoing an echocardiographic assessment of MR. In patients referred for mitral valve surgery, testing was repeated postoperatively.

RESULTS

Echocardiography identified 13 patients with mild MR, 14 with moderate MR, and 26 with severe MR. Among patients with mild, moderate or severe MR, loss of the highest molecular weight von Willebrand factor (VWF) multimers occurred in 8%, 64%, and 85%, respectively, median platelet function analyzer collagen ADP closure times (PFA-CADPs) were 84 s (interquartile range [IQR] 73-96 s), 156 s (IQR 104-181 s), and 190 s (IQR 157-279 s), respectively, and the ratios of VWF latex activity to antigen were 0.92 (IQR 0.83-0.97), 0.85 (IQR 0.76-0.89), and 0.79 (IQR 0.75-0.82), respectively (all P < 0.001). Nine patients reported clinically significant bleeding, and seven had intestinal angiodysplasia and transfusion-dependent gastrointestinal bleeding (Heyde syndrome), with the median number of transfusions required being 20 (IQR 10-33; range 4-50). In patients who underwent mitral valve repair (n = 13) or replacement (n = 7), all measures of VWF function reported above improved significantly.

CONCLUSION

The high-shear environment of moderate to severe MR is sufficient to produce prevalent perturbations in VWF activity. Acquired von Willebrand syndrome may occur in this setting, and appears to be reversible with mitral valve surgery.

Authors+Show Affiliations

Division of Cardiovascular Diseases, Mayo Clinic, Jacksonville, FL, USA.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 available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

25251907

Citation

Blackshear, J L., et al. "Shear Stress-associated Acquired Von Willebrand Syndrome in Patients With Mitral Regurgitation." Journal of Thrombosis and Haemostasis : JTH, vol. 12, no. 12, 2014, pp. 1966-74.
Blackshear JL, Wysokinska EM, Safford RE, et al. Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. J Thromb Haemost. 2014;12(12):1966-74.
Blackshear, J. L., Wysokinska, E. M., Safford, R. E., Thomas, C. S., Shapiro, B. P., Ung, S., ... Chen, D. (2014). Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. Journal of Thrombosis and Haemostasis : JTH, 12(12), pp. 1966-74. doi:10.1111/jth.12734.
Blackshear JL, et al. Shear Stress-associated Acquired Von Willebrand Syndrome in Patients With Mitral Regurgitation. J Thromb Haemost. 2014;12(12):1966-74. PubMed PMID: 25251907.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. AU - Blackshear,J L, AU - Wysokinska,E M, AU - Safford,R E, AU - Thomas,C S, AU - Shapiro,B P, AU - Ung,S, AU - Stark,M E, AU - Parikh,P, AU - Johns,G S, AU - Chen,D, Y1 - 2014/10/17/ PY - 2014/04/07/received PY - 2014/09/17/accepted PY - 2014/9/25/entrez PY - 2014/9/25/pubmed PY - 2015/9/4/medline KW - gastrointestinal hemorrhage KW - hemorrhage KW - mitral valve insufficiency KW - von Willebrand diseases KW - von Willebrand factor SP - 1966 EP - 74 JF - Journal of thrombosis and haemostasis : JTH JO - J. Thromb. Haemost. VL - 12 IS - 12 N2 - BACKGROUND: Mitral valve regurgitation is associated with an acquired hemostatic defect. OBJECTIVE: We sought to assess the prevalence and severity of acquired von Willebrand syndrome in patients with native valve mitral regurgitation (MR). PATIENTS/METHODS: Fifty-three patients were prospectively observed with bleeding questionnaires and laboratory tests when undergoing an echocardiographic assessment of MR. In patients referred for mitral valve surgery, testing was repeated postoperatively. RESULTS: Echocardiography identified 13 patients with mild MR, 14 with moderate MR, and 26 with severe MR. Among patients with mild, moderate or severe MR, loss of the highest molecular weight von Willebrand factor (VWF) multimers occurred in 8%, 64%, and 85%, respectively, median platelet function analyzer collagen ADP closure times (PFA-CADPs) were 84 s (interquartile range [IQR] 73-96 s), 156 s (IQR 104-181 s), and 190 s (IQR 157-279 s), respectively, and the ratios of VWF latex activity to antigen were 0.92 (IQR 0.83-0.97), 0.85 (IQR 0.76-0.89), and 0.79 (IQR 0.75-0.82), respectively (all P < 0.001). Nine patients reported clinically significant bleeding, and seven had intestinal angiodysplasia and transfusion-dependent gastrointestinal bleeding (Heyde syndrome), with the median number of transfusions required being 20 (IQR 10-33; range 4-50). In patients who underwent mitral valve repair (n = 13) or replacement (n = 7), all measures of VWF function reported above improved significantly. CONCLUSION: The high-shear environment of moderate to severe MR is sufficient to produce prevalent perturbations in VWF activity. Acquired von Willebrand syndrome may occur in this setting, and appears to be reversible with mitral valve surgery. SN - 1538-7836 UR - https://www.unboundmedicine.com/medline/citation/25251907/Shear_stress_associated_acquired_von_Willebrand_syndrome_in_patients_with_mitral_regurgitation_ L2 - https://doi.org/10.1111/jth.12734 DB - PRIME DP - Unbound Medicine ER -