PB0826 - A Systematic Review of Acquired von Willebrand Syndrome Associated With Cardiac Disorders: Epidemiology, Disease Burden, and Management

Background: Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder, which can be caused by certain cardiac disorders. High blood shear rates specific to these disorders are associated with loss of high molecular weight multimers of von Willebrand factor (VWF), which impairs VWF function.

Aims: To describe the epidemiology, disease burden, and management of cardiac-related aVWS.

Methods: A systematic review (PROSPERO 2021 CRD42021241527) of studies/reports published up to 31 January 2021 (no date limit) was performed using MEDLINE, Cochrane Central Register for Controlled Trials, EMBASE, and Web of Science databases utilizing keywords encompassing aVWS and cardiac conditions. Additional searches were performed using trial registries, grey literature (ethos.bl.uk, dart-europe.eu, oatd.org), congresses, and Google Scholar. Identified studies were screened by 2 independent reviewers. Review articles and duplicates were removed, and data from identified publications extracted.

Results: Of 70 studies included, 37 were cohort studies, 24 case studies/series, 7 case-control studies, and 2 device experimental studies. Cumulative sample size was 2482 patients with cardiac conditions; median (range) age was 49 (1–89) years at aVWS diagnosis. aVWS occurred frequently in patients with cardiac failure on ventricular assist devices, mechanical circulatory support, or extracorporeal membrane oxygenation/life support (53–100% of patients; 34 cohort studies), or with aortic stenosis (8 cohort studies; patient % not available). Bleeding-related events/complications (mostly gastrointestinal and intracranial bleeding, hemothorax, and epistaxis) were reported in 3–100% of patients (29 cohort studies). Deaths were reported in 16 cohort studies and 4 case reports (407/1378 patients); most common reasons were multiple organ failure (n=24), cardiomyopathy (n=10), and serious bleeding events (n=8). Reported treatments primarily targeted underlying conditions and control of bleeding and related complications; VWF concentrates/factor VIII were rarely used (Table 1).

Conclusion(s): This systematic review highlights the limited data on aVWS epidemiology and disease burden and indicates the complexities of managing patients with cardiac-related aVWS.