Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation

J. L. Blackshear, E. M. Wysokinska, R. E. Safford, Thomas Christopher Smyrk, Brian P Shapiro, S. Ung, M. E. Stark, P. Parikh, G. S. Johns, D. Chen

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1966-1974
Number of pages9
JournalJournal of Thrombosis and Haemostasis
Volume12
Issue number12
DOIs
StatePublished - Dec 1 2014

Fingerprint

Mitral Valve Insufficiency
von Willebrand Factor
Mitral Valve
Hemorrhage
Angiodysplasia
Latex
Hemostatics
Adenosine Diphosphate
Echocardiography
Collagen
Blood Platelets
Molecular Weight
Antigens

Keywords

  • Gastrointestinal hemorrhage
  • Hemorrhage
  • Mitral valve insufficiency
  • Von Willebrand diseases
  • Von Willebrand factor

ASJC Scopus subject areas

  • Hematology

Cite this

Blackshear, J. L., Wysokinska, E. M., Safford, R. E., Smyrk, T. C., 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, 12(12), 1966-1974. https://doi.org/10.1111/jth.12734

Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. / Blackshear, J. L.; Wysokinska, E. M.; Safford, R. E.; Smyrk, Thomas Christopher; Shapiro, Brian P; Ung, S.; Stark, M. E.; Parikh, P.; Johns, G. S.; Chen, D.

In: Journal of Thrombosis and Haemostasis, Vol. 12, No. 12, 01.12.2014, p. 1966-1974.

Research output: Contribution to journalArticle

Blackshear, JL, Wysokinska, EM, Safford, RE, Smyrk, TC, Shapiro, BP, Ung, S, Stark, ME, Parikh, P, Johns, GS & Chen, D 2014, 'Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation', Journal of Thrombosis and Haemostasis, vol. 12, no. 12, pp. 1966-1974. https://doi.org/10.1111/jth.12734
Blackshear, J. L. ; Wysokinska, E. M. ; Safford, R. E. ; Smyrk, Thomas Christopher ; Shapiro, Brian P ; Ung, S. ; Stark, M. E. ; Parikh, P. ; Johns, G. S. ; Chen, D. / Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. In: Journal of Thrombosis and Haemostasis. 2014 ; Vol. 12, No. 12. pp. 1966-1974.
@article{90624f4a21244949a4813c0468e54f89,
title = "Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation",
abstract = "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.",
keywords = "Gastrointestinal hemorrhage, Hemorrhage, Mitral valve insufficiency, Von Willebrand diseases, Von Willebrand factor",
author = "Blackshear, {J. L.} and Wysokinska, {E. M.} and Safford, {R. E.} and Smyrk, {Thomas Christopher} and Shapiro, {Brian P} and S. Ung and Stark, {M. E.} and P. Parikh and Johns, {G. S.} and D. Chen",
year = "2014",
month = "12",
day = "1",
doi = "10.1111/jth.12734",
language = "English (US)",
volume = "12",
pages = "1966--1974",
journal = "Journal of Thrombosis and Haemostasis",
issn = "1538-7933",
publisher = "Wiley-Blackwell",
number = "12",

}

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 - Smyrk, Thomas Christopher

AU - Shapiro, Brian P

AU - Ung, S.

AU - Stark, M. E.

AU - Parikh, P.

AU - Johns, G. S.

AU - Chen, D.

PY - 2014/12/1

Y1 - 2014/12/1

N2 - 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.

AB - 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.

KW - Gastrointestinal hemorrhage

KW - Hemorrhage

KW - Mitral valve insufficiency

KW - Von Willebrand diseases

KW - Von Willebrand factor

UR - http://www.scopus.com/inward/record.url?scp=84916641644&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84916641644&partnerID=8YFLogxK

U2 - 10.1111/jth.12734

DO - 10.1111/jth.12734

M3 - Article

VL - 12

SP - 1966

EP - 1974

JO - Journal of Thrombosis and Haemostasis

JF - Journal of Thrombosis and Haemostasis

SN - 1538-7933

IS - 12

ER -