Acquired von Willebrand syndrome in adult patients with congenital heart disease

•Systematic evaluation of acquired von Willebrand syndrome in congenital heart disease.•We found a high frequency of acquired von Willebrand syndrome.•Preoperative screening for acquired von Willebrand syndrome is recommended.

ObjectivesPostoperative 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).MethodsWe 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.ResultsA 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.ConclusionsThis 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.