Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit.

Background:Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit.Methods:Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children’s National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis.Results:Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support. All patients identified manifested persistent severe bleeding, despite appropriate management of anticoagulation and blood product administration based on the established protocols. Detailed hemostatic testing including quantitative von Willebrand factor (vWF) multimer analysis revealed decreased high-molecular-weight multimers (HMWMs) and absent ult...