Impact of the Severity of Acquired von Willebrand Syndrome on the Short-Term Prognosis in Patients with Temporary Mechanical Circulatory Support

获得性血管性血友病严重程度对接受临时机械循环支持患者短期预后的影响

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Abstract

Background and Objectives: Acquired von Willebrand syndrome (AVWS) develops not only in patients with durable ventricular assist devices but also in patients receiving temporary mechanical circulatory support (MCS). However, its prognostic implication remains unknown. Materials and Methods: Patients who received temporary MCS in our institute between August 2018 and September 2021 were included in this prospective study and the von Willebrand factor multimer analyses were performed following the initiation of temporary MCS supports. The von Willebrand factor large-multimer index was calculated as a normalized ratio of large-multimer proportion among total von Willebrand factor. Association between the large-multimer index and the 30-day survival was investigated. Results: A total of 31 patients (69 years old, 52% men) were included. Median large-multimer index was 63.0% (56.9%, 75.6%). The index was lowest in patients with extracorporeal membrane oxygenation than those receiving support from other devices. A lower index (<59.9%) was associated with lower 30-day survival (41.7% versus 94.7%, p = 0.001) with an odds ratio 0.044 (95% confidence interval 0.002-0.805, p = 0.035) adjusted for other potential confounders. Conclusions: An advanced AVWS was associated with lower short-term survival in patients with temporary MCS. The clinical implication of AVWS-guided temporary MCS management remains the next concern.

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