title: Acquired von Willebrand syndrome in adult patients with congenital heart disease.
abstract: OBJECTIVE
  Postoperative bleeding is common in patients with congenital heart disease (CHD). However, little is known about the role and prevalence of acquired von Willebrand syndrome (AVWS).
  
  
  METHODS
  We evaluated the prevalence of AVWS in relation to underlying cardiac defects, operative procedures and the presence of Eisenmenger syndrome. The prothrombin time, aPTT, platelet function analysis (PFA-100), von Willebrand factor antigen (VWF:Ag), VWF activity, VWF collagen binding activity (VWF:CB), factor VIII activity and multimeric analysis were measured in addition to tests evaluating heart, liver and kidney functions.
  
  
  RESULTS
  A total of 221 patients were screened and 192 patients were included in the study. The overall prevalence of AVWS was 20.8%. AVWS was identified across all of the cardiac defects, with the highest prevalence in the defects of great complexity (38.6% compared to 9.4% in patients with CHD of simple/moderate complexity, p<0.001), Eisenmenger syndrome (p<0.001) and more severe heart failure symptoms (NYHA III/IV vs. NYHA I, p<0.001; NYHA III/IV vs. NYHA II, p=0.044). A combination of multimeric analysis, VWF:CB to VWF:Ag ratio (sensitivity: 77.5%, specificity: 93.3%) and PFA-100 (PFA Col/Epi sens.: 77%, spec.: 52%; PFA Col/ADP sens.: 75%, spec.: 74.3%) were used to detect AVWS.
  
  
  CONCLUSIONS
  This study demonstrated that AVWS occurred in patients with various congenital cardiac defects, but the highest prevalence occurred in the patients with complex CHD and Eisenmenger syndrome. We, therefore, suggest preoperative screening for AVWS in all of the patients with CHD, particularly in the patients with CHD of greater complexity and suffering from Eisenmenger syndrome.
authors.affiliation: Werlhof-Institute Hannover, Schillerstraße 23, Hannover D-30159, Germany. Electronic address: HCW@werlhof-institut.de.
authors.forename: Hans Christian
authors.initials: HC
authors.lastname: Waldow
authors.affiliation: Department of Cardiovascular Medicine, Hannover Medical School, Hannover, Germany.
authors.forename: Mechthild
authors.initials: M
authors.lastname: Westhoff-Bleck
authors.affiliation: Department of Cardiovascular Medicine, Hannover Medical School, Hannover, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Widera
authors.affiliation: Cardiovascular Centre, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Christian
authors.initials: C
authors.lastname: Templin
authors.affiliation: Werlhof-Institute Hannover, Schillerstraße 23, Hannover D-30159, Germany.
authors.forename: Mario
authors.initials: M
authors.lastname: von Depka
mesh_terms: D000328:Adult; D000368:Aged; D001780:Blood Coagulation Tests / Q000379:methods; D005260:Female; D006330:Heart Defects, Congenital / Q000097:blood* / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D011517:Prothrombin Time / Q000379:methods; D055815:Young Adult; D014842:von Willebrand Diseases / Q000097:blood* / Q000175:diagnosis / Q000209:etiology*
keywords: Acquired von Willebrand syndrome; Bleeding; Congenital heart disease; Eisenmenger syndrome