Desmopressin (DDAVP) use in patients with von Willebrand disease: A single‐centre retrospective review of test response and clinical outcomes

Abstract

AbstractIntroductionPeri‐procedural management of von Willebrand disease (VWD) utilizes von Willebrand factor (VWF) concentrates or desmopressin (DDAVP) to increase VWF levels. DDAVP is safe, easily administered, and inexpensive. Currently, a consensus definition for adequate DDAVP response is lacking, and outcomes of peri‐procedural DDAVP use in VWD patients are seldom reported.AimThis single‐centre retrospective review aims to characterize DDAVP‐responsiveness and assess clinical outcomes of peri‐procedural DDAVP use in VWD.Patients and MethodsWe reviewed records for all our adult VWD patients (age ≥18 years) who underwent DDAVP challenge testing between January 2007 and January 2022. DDAVP‐responsiveness was assessed using six definitions. Bleeding outcomes following procedures covered by DDAVP were classified as excessive or expected bleeding.ResultsEighty‐four of 94 (89.4%) patients were DDAVP‐responsive by our definition (1‐h VWF Activity/Factor VIII ≥0.50 IU/mL). However, the proportion of DDAVP‐responders varied from 53.2% to 91.5%, depending on the literature definition used. Ninety‐nine procedures pre‐treated with DDAVP were performed during the study period. Eighty‐six (86.7%) procedures (31 major; 55 minor) were covered with only DDAVP ± tranexamic acid (TXA). Excessive bleeding occurred following 4/31 major procedures and 2/55 minor procedures (both performed in a single patient with a bleeding score of 16). When covered with DDAVP+Factor ± TXA, one each of 10 major and 3 minor procedures (performed in 2 patients with bleeding scores 15–16) resulted in post‐procedural bleeding.ConclusionsPeri‐procedural DDAVP prophylaxis appears to be effective among individuals with VWD. Beyond DDAVP‐responsiveness, patient bleeding history and procedure invasiveness should be considered in determining suitability for DDAVP prophylaxis.