Perioperative management of transcatheter aortic valve implantation in acquired von Willebrand syndrome secondary to monoclonal gammopathy: a case report.

Abstract

Background: Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder associated with conditions like monoclonal gammopathy of undetermined significance (MGUS). It results from monoclonal antibodies binding to von Willebrand factor (vWF), leading to rapid clearance of vWF and factor VIII (FVIII). Of note, any condition increasing shear stress, such as aortic stenosis (AS), can exacerbate AvWS.

Case summary: We report the case of an 81-year-old man with AvWS secondary to MGUS who underwent transcatheter aortic valve implantation (TAVI) for severe aortic stenosis. Pre-operative intravenous immunoglobulins (IVIG) were administered, which normalized FVIII and vWF levels. TAVI was performed without specific haemostatic measures other than vascular closure devices. There were no bleeding complications, and the patient was discharged without antiplatelet therapy. At one-year follow-up, he had no thrombotic or haemorrhagic events.

Discussion: We report the management of a patient affected by AvWS secondary to MGUS who underwent TAVI for severe AS. IVIG were administered preoperatively to temporarily normalize coagulation, as their mechanism of action directly inhibits the increased consumption of vWF. Due to the increased bleeding risk associated with AvWS, antiplatelet therapy was not initiated upon discharge.