title: Treatment of acquired von Willebrand syndrome in aortic stenosis with transcatheter aortic valve replacement.
abstract: OBJECTIVE
  This study sought to investigate the prevalence of abnormal von Willebrand multimers (AbM) in patients undergoing transcatheter aortic valve replacement (TAVR) and the impact of TAVR on the underlying factor variances.
  
  
  BACKGROUND
  An association between the acquired von Willebrand syndrome (aVWS) and valvular aortic stenosis (AS) has been established in the past and surgical aortic valve replacement (SAVR) shown to lead to factor recovery. Prevalence and course of AbM in patients treated with TAVR though has not yet been described comprehensively.
  
  
  METHODS
  Ninety-five consecutive patients underwent TAVR at our institution. Hemostaseologic testing was performed before and up to 1 week after TAVR. Transvalvular and right heart hemodynamics as well as bleeding episodes were recorded and analyzed with descriptive statistics.
  
  
  RESULTS
  Baseline prevalence of AbM was 42% with an average high-molecular-weight multimer (HMWM) count of 16.2 ± 3.3%. Pressure gradients correlated significantly with the extent of HMWM deficiency (r = -0.63 [p < 0.0001]). Following valve implantation, HMWM increased proportional to the drop in mean pressure gradient and normalized in most of the patients. However, residual aortic regurgitation/leakage led to inferior HMWM recovery but prosthesis-patient mismatch (PPM) was rare and left HMWM uninfluenced. We saw no association of transfusion with AbM and 1-year mortality was unaffected by AbM.
  
  
  CONCLUSIONS
  AbM in patients with AS undergoing TAVR is frequent. However, TAVR is capable of correcting AbM and therefore possibly aVWS in patients with AS. As opposed to SAVR, bleeding and transfusion requirement in TAVR patients was not associated with severe HMWM deficiency; PPM was rare and HMWM were uninfluenced by the procedure. Aortic regurgitation after TAVR adversely influenced HMWM recovery.
authors.affiliation: Division of Cardiology, Asklepios Klinik St. Georg Hospital, Hamburg, Germany. Electronic address: t.spangenberg@asklepios.com.
authors.forename: Tobias
authors.initials: T
authors.lastname: Spangenberg
authors.affiliation: Medilys Laborgesellschaft mbH, Hemostaseology, Hamburg, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Budde
authors.affiliation: Division of Cardiology, Asklepios Klinik St. Georg Hospital, Hamburg, Germany.
authors.forename: Dimitry
authors.initials: D
authors.lastname: Schewel
authors.affiliation: Division of Cardiology, Asklepios Klinik St. Georg Hospital, Hamburg, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Frerker
authors.affiliation: Division of Cardiology, Asklepios Klinik St. Georg Hospital, Hamburg, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Thielsen
authors.affiliation: Division of Cardiology, Asklepios Klinik St. Georg Hospital, Hamburg, Germany.
authors.forename: Karl-Heinz
authors.initials: KH
authors.lastname: Kuck
authors.affiliation: Division of Cardiology, Asklepios Klinik St. Georg Hospital, Hamburg, Germany; Division of Cardiology, University Heart Center Eppendorf, Hamburg, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Schäfer
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001022:Aortic Valve Insufficiency / Q000175:diagnosis / Q000453:epidemiology; D001024:Aortic Valve Stenosis / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*; D006328:Cardiac Catheterization / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods*; D005260:Female; D005858:Germany / Q000453:epidemiology; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods*; D006439:Hemodynamics; D006801:Humans; D008297:Male; D015995:Prevalence; D012042:Registries; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*
keywords: aortic stenosis; transcatheter aortic valve replacement; von Willebrand syndrome