Abstract 20298: Acquired Von Willebrand Syndrome in Patients With Hypertrophic Cardiomyopathy: A Case Series
Introduction: von Willebrand factor (vWF) is a multimeric glycoprotein involved in hemostasis. Acquired von Willebrand Syndrome (AvWS) results from a disproportionate loss of high molecular weight multimers of vWF, and leads to increased risk of bleeding. Hypertrophic cardiomyopathy (HCM) has been implicated as a cause of AvWS due to turbulent flow and shear stress due to left ventricular outflow tract obstruction and from associated mitral regurgitation.
Methods: This is a retrospective study in patients with HCM and gastrointestinal (GI) bleeding at a tertiary referral HCM clinic from 2000-2013. Patients were selected from a HCM database and cross referenced with medical record searches for GI bleed and vWF. All patients had echocardiographic evidence of HCM and measurement of hemoglobin. AvWS was based on measurements of vWF multimer analysis.
Results: Of the eight patients who met criteria with HCM and GI bleed or anemia, four underwent assessment for AvWS prior to intervention (Table 1). Mean age at AvWS diagnosis was 63 years (2 females). Two patients demonstrated improvement in anemia post-myectomy; vWF were not retested. One patient is scheduled for septal myectomy given continued anemia. The fourth had resolution of bleeding with medical management alone.
Four additional patients were identified but not evaluated for AvWS. Three patients underwent septal myectomy with notable resolution of anemia. One patient has ongoing bleed in the setting of multiple known GI angiectasias
Conclusions: Despite prior published data linking severe bleeding to HCM secondary to AvWS, only half of patients with GI bleeding at our institution underwent assessment for AvWS. Given a favorable response to myectomy, HCM patients with GI bleeding warrant further investigation, as diagnosis of AvWS may alter treatment plans.
Author Disclosures: A.J. McCambridge: None. E.R. Fenstad: None. J.B. Geske: None. J.L. Blackshear: None. S.R. Ommen: None.
- © 2014 by American Heart Association, Inc.