Management of Elective Procedures in Low von Willebrand factor patients in the LoVIC study

Authors: Doherty, D., Lavin, M., O’Sullivan, JM., Ryan, K., O’Connell, NM., Dougall, A., Byrne, M., Rafferty, M., Doyle, MM., Di, Paola J., James, P., and O’Donnell, JS.

Publication: J. ThrombThromb . Haemost Haemost HaemostHaemost. January 2021

Affiliations: National Coagulation Centre, St James’s Hospital, Dublin, Ireland; Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland; Department of Pediatrics, Washington University School of Medicine in St. Louis, St. Louis, Missouri, USA; Department of Medicine, Queen’s University, Kingston, ON, Canada ; National Children’s Research Centre, Our Lady’s Children’s Hospital Crumlin, Dublin, Ireland.

Abstract: BACKGROUND: Most individuals with mild to moderate reductions in plasma VWF levels do not demonstrate increased bleeding. However, some patients with plasma VWF levels in the 30-50 IU/dL do have a significant bleeding phenotype. Management of these ‘Low VWF’ patients, who may have significant bleeding scores > 10, around times of elective procedures continues to pose a common clinical challenge due to lack of evidence. OBJECTIVE: To investigate the safety and efficacy of different peri-procedural management options for adult patients with Low VWF. METHODS: Treatment and outcomes were retrospectively reviewed for 160 invasive procedures performed in 60 patients with well characterized Low VWF enrolled in the previously described Low von Willebrand factor Ireland Cohort (LoVIC) study. RESULTS: We demonstrate that DDAVP is efficacious in preventing bleeding for both minor and major elective procedures in adult Low VWF patients, even in those with significant bleeding histories. In addition, tranexamic acid alone is effective for Low VWF patients undergoing non-dental minor procedures. Importantly, age-related increases in plasma VWF:Ag levels above 50 IU/dL were not necessarily associated with complete correction of bleeding phenotype. Procedure-related bleeding complications were increased in Low VWF patients who did not receive any haemostatic cover prior to their procedure. CONCLUSION: Elective procedures in adult patients with Low VWF should be managed in liaison with a Comprehensive Care tertiary referral centre so that personalized treatment plans may be implemented prior to all minor or major elective procedures.