Quality of periprocedural care in patients with von Willebrand disease.

INTRODUCTION: While it has been shown that haemophilia patients receiving care at Haemophilia Treatment Centres (HTCs) experience decreased morbidity and mortality, little research has been done on the outcomes of patients with von Willebrand disease (VWD). AIM: To compare the quality of periprocedural care received by patients with VWD at HTCs and non-HTCs. METHODS: We performed a retrospective chart review on all adult VWD patients undergoing an invasive procedure from 2015 to 2017. Quality of periprocedural care was measured using the following surrogate outcomes: periprocedural VWD-specific therapy use per 2007 National Heart, Lung, and Blood Institute (NHLBI) guidelines, procedural estimated blood loss (EBL), and post-procedure bleeding. Comparisons were performed according to the setting of care at the time of the invasive procedure, HTC versus non-HTC. RESULTS: There were 668 invasive procedures performed on 305 patients, of which 8.2% were HTC cases. Non-type 1 VWD was more likely in HTC cases. VWD-specific therapy was used per NHLBI guidelines in 100% of HTC cases compared with 10.6% of non-HTC cases. Procedural EBL > = 100 ml was more likely to occur in HTC differences cases (OR = 2.34; 95% CI, 1.05 to 5.25). There was no difference in post-procedure bleeding between the two groups (OR = 1.26, 95% CI, .20- 7.86). CONCLUSION: Despite widespread periprocedural use of VWD-specific therapy outside established guidelines at non-HTCs, there was no difference in periprocedural bleeding. Possible explanations include diagnostic error, in disease severity and procedure types, and dataset limitations. Additional studies are needed to investigate this further and compare other patient care outcomes between HTCs and non-HTCs.