Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy
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...
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| Veröffentlicht in: | The American journal of cardiology 2016-02, Vol.117 (3), p.436-442 |
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| creator | Blackshear, Joseph L., MD Kusumoto, Hana Safford, Robert E., MD, PhD Wysokinska, Ewa, MD Thomas, Colleen S., MS Waldo, Oral A., MD Stark, Mark E., MD Shapiro, Brian P., MD Ung, Steven, MD Moussa, Issam, MD Agnew, Richard C., MD Landolfo, Kevin, MD Chen, Dong, MD, PhD |
| description | 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 |
| doi_str_mv | 10.1016/j.amjcard.2015.11.016 |
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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.</description><identifier>ISSN: 0002-9149</identifier><identifier>EISSN: 1879-1913</identifier><identifier>DOI: 10.1016/j.amjcard.2015.11.016</identifier><identifier>PMID: 26705879</identifier><identifier>CODEN: AJCDAG</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Adult ; Aged ; Antigens ; Blood platelets ; Brain research ; Cardiac Surgical Procedures - methods ; Cardiology ; Cardiomyopathy ; Cardiomyopathy, Hypertrophic - blood ; Cardiomyopathy, Hypertrophic - physiopathology ; Cardiomyopathy, Hypertrophic - surgery ; Cardiovascular ; Collagen ; Echocardiography ; Female ; Follow-Up Studies ; Heart Septum - surgery ; Humans ; Male ; Middle Aged ; Molecular weight ; Patients ; Peptides ; Prognosis ; Retrospective Studies ; Variables ; Ventricular Function, Left - physiology ; von Willebrand Factor - metabolism</subject><ispartof>The American journal of cardiology, 2016-02, Vol.117 (3), p.436-442</ispartof><rights>Elsevier Inc.</rights><rights>2016 Elsevier Inc.</rights><rights>Copyright © 2016 Elsevier Inc. All rights reserved.</rights><rights>Copyright Elsevier Limited Feb 1, 2016</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c584t-5c8fd5fe46eb296e3adcbbd71aba400262d0978a07623805ad402393cbff1e793</citedby><cites>FETCH-LOGICAL-c584t-5c8fd5fe46eb296e3adcbbd71aba400262d0978a07623805ad402393cbff1e793</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.proquest.com/docview/1759025911?pq-origsite=primo$$EHTML$$P50$$Gproquest$$H</linktohtml><link.rule.ids>315,781,785,3551,27929,27930,46000,64390,64392,64394,72474</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26705879$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Blackshear, Joseph L., MD</creatorcontrib><creatorcontrib>Kusumoto, Hana</creatorcontrib><creatorcontrib>Safford, Robert E., MD, PhD</creatorcontrib><creatorcontrib>Wysokinska, Ewa, MD</creatorcontrib><creatorcontrib>Thomas, Colleen S., MS</creatorcontrib><creatorcontrib>Waldo, Oral A., MD</creatorcontrib><creatorcontrib>Stark, Mark E., MD</creatorcontrib><creatorcontrib>Shapiro, Brian P., MD</creatorcontrib><creatorcontrib>Ung, Steven, MD</creatorcontrib><creatorcontrib>Moussa, Issam, MD</creatorcontrib><creatorcontrib>Agnew, Richard C., MD</creatorcontrib><creatorcontrib>Landolfo, Kevin, MD</creatorcontrib><creatorcontrib>Chen, Dong, MD, PhD</creatorcontrib><title>Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy</title><title>The American journal of cardiology</title><addtitle>Am J Cardiol</addtitle><description>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.</description><subject>Adult</subject><subject>Aged</subject><subject>Antigens</subject><subject>Blood platelets</subject><subject>Brain research</subject><subject>Cardiac Surgical Procedures - methods</subject><subject>Cardiology</subject><subject>Cardiomyopathy</subject><subject>Cardiomyopathy, Hypertrophic - blood</subject><subject>Cardiomyopathy, Hypertrophic - physiopathology</subject><subject>Cardiomyopathy, Hypertrophic - surgery</subject><subject>Cardiovascular</subject><subject>Collagen</subject><subject>Echocardiography</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Heart Septum - surgery</subject><subject>Humans</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Molecular weight</subject><subject>Patients</subject><subject>Peptides</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><subject>Variables</subject><subject>Ventricular Function, Left - 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Academic</collection><jtitle>The American journal of cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Blackshear, Joseph L., MD</au><au>Kusumoto, Hana</au><au>Safford, Robert E., MD, PhD</au><au>Wysokinska, Ewa, MD</au><au>Thomas, Colleen S., MS</au><au>Waldo, Oral A., MD</au><au>Stark, Mark E., MD</au><au>Shapiro, Brian P., MD</au><au>Ung, Steven, MD</au><au>Moussa, Issam, MD</au><au>Agnew, Richard C., MD</au><au>Landolfo, Kevin, MD</au><au>Chen, Dong, MD, PhD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy</atitle><jtitle>The American journal of cardiology</jtitle><addtitle>Am J Cardiol</addtitle><date>2016-02-01</date><risdate>2016</risdate><volume>117</volume><issue>3</issue><spage>436</spage><epage>442</epage><pages>436-442</pages><issn>0002-9149</issn><eissn>1879-1913</eissn><coden>AJCDAG</coden><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.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>26705879</pmid><doi>10.1016/j.amjcard.2015.11.016</doi><tpages>7</tpages></addata></record> |
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| subjects | Adult Aged Antigens Blood platelets Brain research Cardiac Surgical Procedures - methods Cardiology Cardiomyopathy Cardiomyopathy, Hypertrophic - blood Cardiomyopathy, Hypertrophic - physiopathology Cardiomyopathy, Hypertrophic - surgery Cardiovascular Collagen Echocardiography Female Follow-Up Studies Heart Septum - surgery Humans Male Middle Aged Molecular weight Patients Peptides Prognosis Retrospective Studies Variables Ventricular Function, Left - physiology von Willebrand Factor - metabolism |
| title | Usefulness of Von Willebrand Factor Activity Indexes to Predict Therapeutic Response in Hypertrophic Cardiomyopathy |
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