Perioperative management for patients with von Willebrand disease: Defining the optimal approach

Abstract

von Willebrand disease (VWD) is the most common inherited bleeding disorder characterised by a quantitative or qualitative deficiency in von Willebrand factor (VWF). During invasive surgical procedures, patients with VWD require additional treatment to maintain haemostasis; however, due to the complexity of VWD, there is a lack of consensus on the optimal management. In the perioperative period, patients are usually treated with VWF and factor FVIII (FVIII)‐containing concentrates to provide an immediate haemostatic response to prevent excessive bleeding during both elective and emergency surgery. With the introduction of recombinant VWF (rVWF), there is a need for guidance on the use of the various VWF products in the perioperative period for all types of patients and surgeries. This review provides an overview of the current evidence for the surgical management of patients with VWD and, summarises the optimal treatment approach during the perioperative period, and highlights key unanswered questions and the research needed to address the evidence gaps.

Management of von Willebrand disease. Recommendations of the Hemostasis Group of the Polish Society of Hematology and Transfusiology (2022)

“…Transient thrombocytopenia may also occur, but is not usually associated with bleeding. In 2B subtype patients, DDAVP may be considered for minor bleedings given the risk of thrombocytopenia [37,38]. In type 3 VWD, DDAVP is not effective [8].…”

“…If required, maintenance/subsequent doses are administered every 12-24 hours. Repetitive administration of the drug (over a period of several days) results in reduced response to therapy, most likely due to depletion of tissue coagulation factors (tachyphylaxis) [38].…”

“…DDAVP is to be avoided during neuro-ophthalmic and cardiovascular procedures because of the reported adverse reactions. Pregnancy is no contraindication for DDAVP, because of its negligible effect on uterus contractility [8,38].…”

“…It is recommended to avoid the maintenance of VWF:RCo and FVIII:C values above 150 IU/dL for longer periods of time in patients at higher risk of thrombosis. Routine antithrombotic prophylaxis should be applied [8,38]. Table 6 lists FVIII/VWF concentrates, (both plasma-derived and recombinant), registered in the European Union (as for 01.2022).…”

“…FVIII/VWF factor concentrates approved in the European Union for VWD therapy (as for 01.2022)[6,38,40,41] …”