Current management of von Willebrand disease and von Willebrand syndrome

Anesthesiologists frequently care for patients with altered hemostasis and coagulation. Where a clear history of familial and personal bleeding exists, a thoughtful plan can be developed in advance to manage the issue perioperatively. However, in some cases, it may not be known that the patient has a disorder until excessive bleeding is noted during or after surgery. Recognition of the issue and appropriate targeted therapy are the keys to successful management. With an estimated prevalence approaching 1% of the population, von Willebrand disease (vWD) is the most common hereditary bleeding diathesis, but the estimated prevalence of acquired vWD (often termed von Willebrand syndrome or vWS) is now believed to be significantly higher, especially in patients with malignancies, autoimmune diseases, cardiac valvular lesions, and in patients on mechanical circulatory support devices. Acquired vWD may also occur with certain medications. The mainstay of the diagnosis of vWD is laboratory testing. Preoperative clinical assessment and a high level of suspicion are often effective to alert the anesthesiologist to the possibility of vWS, thus allowing for appropriate testing and potential prophylaxis in elective situations, as well as appropriately targeted therapy of unexpected bleeding when a hemostatic derangement was not anticipated preoperatively.