TITLE

Hypertrophic Obstructive Cardiomyopathy, Bleeding History, and Acquired von Willebrand Syndrome: Response to Septal Myectomy

AUTHOR(S)
BLACKSHEAR, JOSEPH L.; SCHAFF, HARTZELL V.; OMMEN, STEVE R.; DONG CHEN; NICHOLS, WILLIAM L.
PUB. DATE
March 2011
SOURCE
Mayo Clinic Proceedings;Mar2011, Vol. 86 Issue 3, p219
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Bleeding with severe aortlc stenosis is linked to acquired von Wiliebrand syndrome and loss of high-molecular-weight multimers of von Willebrand factor. Valve replacement resolves bleeding tendency and loss of high-molecular-weight muitimers. We report outcomes In 5 patients with symptomatic obstructive hypertrophic cardlomyopathy and spontaneous gastrointestinal, mucosal, or excessive postsurgicai bleeding in whom acquired von Willebrand syndrome was documented. All 5 patients under- went surgical septal myectomy with resolution of acquired von Willebrand syndrome. Mayo Clin Proc. 2011;86(3):219.224
ACCESSION #
60010650

 

Related Articles

  • Effect of Genetic Variation in STXBP5 and STX2 on von Willebrand Factor and Bleeding Phenotype in Type 1 von Willebrand Disease Patients. Van Loon, Janine E.; Sanders, Yvonne V.; De Wee, Eva M.; Kruip, Marieke J. H. A.; De Maat, Moniek P. M.; Leebeek, Frank W. G. // PLoS ONE;Jul2012, Vol. 7 Issue 7, p1 

    Background: In type 1 von Willebrand Disease (VWD) patients, von Willebrand Factor (VWF) levels and bleeding symptoms are highly variable. Recently, the association between genetic variations in STXBP5 and STX2 with VWF levels has been discovered in the general population. We assessed the...

  • Kidney function and multiple hemostatic markers: cross sectional associations in the multi-ethnic study of atherosclerosis. Dubin, Ruth; Cushman, Mary; Folsom, Aaron R.; Fried, Linda F.; Palmas, Walter; Peralta, Carmen A.; Wassel, Christina; Shlipak, Michael G. // BMC Nephrology;2011, Vol. 12 Issue 1, p3 

    Background: Defined as estimated glomerular filtration rate (eGFR) < 60 ml/min/1.73 m2, chronic kidney disease (CKD) is strongly and independently associated with cardiovascular and overall mortality. We hypothesized that reduced kidney function would be characterized by abnormalities of...

  • Genetic Testing for the Diagnosis of Von Willebrand Disease: Benefits and Limitations. Favaloro, Emmanuel J.; Krigstein, Michael; Koutts, Jerry; Brighton, Timothy; Lindeman, Robert // Journal of Coagulation Disorders;2010, Vol. 2 Issue 2, p37 

    von Willebrand disease (VWD) is the most common inherited bleeding disorder and arises from deficiencies and/or defects in the plasma protein von Willebrand factor (VWF). VWD is classified into six different types, with Type 1 identified as a (partial) quantitative deficiency of VWF, Type 3 a...

  • Cleavage of von Willebrand Factor by Granzyme M Destroys Its Factor VIII Binding Capacity. Hollestelle, Martine J.; Lai, Ka Wai; van Deuren, Marcel; Lenting, Peter J.; de Groot, Philip G.; Sprong, Tom; Bovenschen, Niels // PLoS ONE;2011, Vol. 6 Issue 9, p1 

    Von Willebrand factor (VWF) is a pro-hemostatic multimeric plasma protein that promotes platelet aggregation and stabilizes coagulation factor VIII (FVIII) in plasma. The metalloproteinase ADAMTS13 regulates the platelet aggregation function of VWF via proteolysis. Severe deficiency of ADAMTS13...

  • von Willebrand Factor.  // Encyclopedic Reference of Molecular Pharmacology;2004, p1009 

    A definition of the term "von Willebrand Factor" (vWf) is presented. It refers to a heterogeneous multimeric plasma glycoprotein produced by megakaryocytes and endothelial cells which is found in platelets, plasma and the subendothelium. Subendothelial vWf facilitates platelet adhesion,...

  • Relationship between ABO blood group and von Willebrand factor levels: from biology to clinical implications. Franchini, Massimo; Capra, Franco; Targher, Giovanni; Montagnana, Martina; Lippi, Giuseppe // Thrombosis Journal;2007, Vol. 5, p14 

    Although a number of studies have demonstrated the influence of ABO blood group on plasma levels of von Willebrand factor (VWF), the nature of this association and its clinical importance is still largely unknown. In this review, the most recent advances in our understanding of the mechanisms by...

  • Identification and Functional Analysis of a Novel von Willebrand Factor Mutation in a Family with Type 2A von Willebrand Disease. Jing Dong; Xiaojuan Zhao; Sensen Shi; Zhenni Ma; Meng Liu; Qingyu Wu; Changgeng Ruan; Ningzheng Dong // PLoS ONE;Mar2012, Vol. 7 Issue 3, p1 

    von Willebrand factor (VWF) is essential for normal hemostasis. VWF gene mutations cause the hemorrhagic von Willebrand disease (VWD). In this study, a 9-year-old boy was diagnosed as type 2A VWD, based on a history of abnormal bleeding, low plasma VWF antigen and activity, low plasma factor...

  • Von Willebrand Factor Antigen and Factor XI Activity Levels As Predictors of Bleeding Tendency in Israeli Patients with Von Willebrand's Disease. Brenner, Benjamin; Steinberg, Tamar; Laor, Arieh; Tavori, Shulamit; Tatarsky, Ilana; Lanir, Naomi // Clinical & Applied Thrombosis/Hemostasis;Fall1995, Vol. 1 Issue 4, p260 

    Previous preliminary data and case reports have suggested an association of von Willebrand's disease (vWD) with factor XI deficiency and platelet abnormalities. We have analyzed the prevalence of factor XI deficiency and thrombocytopathy in a cohort of Israeli patients with vWD. Decreased factor...

  • A New Automated Screening Assay for the Diagnosis of von Willebrand Disease. Raneem Salem // American Journal of Clinical Pathology;May2007, Vol. 127 Issue 5, p730 

    A new, automated assay for von Willebrand factor (vWF) activity has recently become commercially available (HemosIL vWF activity assay, Instrumentation Laboratories, Lexington, MA). We prospectively studied 61 specimens from 58 patients undergoing laboratory testing for suspicion of von...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics