Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy

Joseph L. Blackshear, Hana Kusumoto, Robert E. Safford, Ewa Wysokinska, Colleen S. Thomas, Oral A. Waldo, Mark E. Stark, Brian P Shapiro, Steven Ung, Issam Moussa, Richard C. Agnew, Kevin Landolfo, Dong Chen

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Degraded by shear stress, loss of high-molecular-weight multimers of von Willebrand factor (VWF) correlates strongly with pressure gradient in aortic stenosis (AS) and obstructive hypertrophic cardiomyopathy (HC). We assessed VWF tests before and after interventions in HC and contrasted the severity of abnormalities in HC to patients with AS, mitral regurgitation, and left ventricular assist devices. Ninety patients with median (interquartile range) age 66 (53 to 72) years, 51% men, with HC had assessments of 3 VWF parameters and B-type natriuretic peptide before and after 26 discreet medical/pacing interventions, 22 alcohol septal ablations, and 28 ventricular septal myectomies. VWF multimers were abnormal in 87% of patients with obstructive HC versus 48% of patients with latent obstruction (p = 0.0001). VWF measurements correlated with peak instantaneous left ventricular outflow tract gradient, Spearman ρ 0.51 to 0.61, p <0.0001. For B-type natriuretic peptide, correlation with left ventricular outflow tract gradient was weaker, ρ = 0.37, p = 0.0005, but stronger with septal thickness or mitral E/e'. In pre-/post-medical treatment of HC, VWF multimers were abnormal in 73%/68% of patients, p = 0.74; pre-/post-septal ablation 74%/26%, p = 0.0035; and pre-/post-septal myectomy 75%/0%, p <0.0001. In obstructive HC, the degree VWF multimer loss was greater than in severe AS or severe mitral regurgitation and overlapped that seen in left ventricular assist devices. In conclusion, VWF activity indexes were predictably abnormal in patients with HC with resting obstruction to a degree where bleeding could be anticipated, accurately reflected gradient changes after intervention, and demonstrated complete normalization after septal myectomy.

Original languageEnglish (US)
Pages (from-to)436-442
Number of pages7
JournalAmerican Journal of Cardiology
Volume117
Issue number3
DOIs
StatePublished - Feb 1 2016

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Hypertrophic Cardiomyopathy
von Willebrand Factor
Aortic Valve Stenosis
Heart-Assist Devices
Brain Natriuretic Peptide
Mitral Valve Insufficiency
Therapeutics
Molecular Weight
Alcohols
Hemorrhage
Pressure

ASJC Scopus subject areas

  • Medicine(all)
  • Cardiology and Cardiovascular Medicine

Cite this

Blackshear, J. L., Kusumoto, H., Safford, R. E., Wysokinska, E., Thomas, C. S., Waldo, O. A., ... Chen, D. (2016). Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy. American Journal of Cardiology, 117(3), 436-442. https://doi.org/10.1016/j.amjcard.2015.11.016

Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy. / Blackshear, Joseph L.; Kusumoto, Hana; Safford, Robert E.; Wysokinska, Ewa; Thomas, Colleen S.; Waldo, Oral A.; Stark, Mark E.; Shapiro, Brian P; Ung, Steven; Moussa, Issam; Agnew, Richard C.; Landolfo, Kevin; Chen, Dong.

In: American Journal of Cardiology, Vol. 117, No. 3, 01.02.2016, p. 436-442.

Research output: Contribution to journalArticle

Blackshear, JL, Kusumoto, H, Safford, RE, Wysokinska, E, Thomas, CS, Waldo, OA, Stark, ME, Shapiro, BP, Ung, S, Moussa, I, Agnew, RC, Landolfo, K & Chen, D 2016, 'Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy', American Journal of Cardiology, vol. 117, no. 3, pp. 436-442. https://doi.org/10.1016/j.amjcard.2015.11.016
Blackshear, Joseph L. ; Kusumoto, Hana ; Safford, Robert E. ; Wysokinska, Ewa ; Thomas, Colleen S. ; Waldo, Oral A. ; Stark, Mark E. ; Shapiro, Brian P ; Ung, Steven ; Moussa, Issam ; Agnew, Richard C. ; Landolfo, Kevin ; Chen, Dong. / Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy. In: American Journal of Cardiology. 2016 ; Vol. 117, No. 3. pp. 436-442.
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