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Introduction: Monitoring changes in symptoms over time during long-term nocturnal home non-invasive ventilation (NIV) using patient-reported outcome measures is crucial. This study aimed to identify factors associated with changes in the S3-NIV total score, its two domains ("respiratory symptoms" and "sleep and NIV-related side effects") and individual item responses., Methods: We conducted a retrospective, longitudinal data analysis of a cohort of adults with chronic respiratory failure treated with NIV. Data were obtained from a French homecare provider. Multivariate linear and multinomial ordinal mixed effect models were used to identify factors associated with changes in S3-NIV scores over time., Results: Median follow-up was 2 years for 2135 participants. Each participant completed a median of five S3-NIV questionnaires; totaling 11,359 analyzed questionnaires. Type of respiratory condition, sex, age and time since NIV initiation were associated with change in S3-NIV score over time. NIV adherence was not associated with total S3-NIV score but high adherence was associated with more severe respiratory symptoms and an improvement in sleep and NIV-related side effects during the follow-up. Intensity of pressure support was associated with a lower total S3-NIV score and more side effects. Face masks and supplemental oxygen were associated with a lower S3-NIV total score., Conclusion: Changes in S3-NIV scores over time are associated with the individual's characteristics and NIV settings. Analysis of the two domains and individual items of the S3-NIV could increase understanding of the difficulties experienced by people on NIV., (Copyright © 2024 SEPAR. Published by Elsevier España, S.L.U. All rights reserved.)

In subjects with neuromuscular diseases (NMD), the choice of facemask is essential for successful long-term noninvasive ventilation (NIV). While nasal masks usually represent the first line of treatment, almost a third of our subjects with NMD use an oro-nasal interface. Factors associated with the choice of mask remain poorly understood. We provide an original analysis of a previous prospective, multi-centric, Franco-Belgian survey investigating the factors associated with the type of nocturnal mask used in 116 adult NMD subjects treated with NIV. In these patients oro-nasal mask use was more often associated with non-Duchenne muscular dystrophy, older subjects, higher body mass index, better upper limb autonomy allowing independent mask removal and shorter periods of ventilation. Controlled prospective studies are needed to compare the efficacy and tolerance of different interfaces in this specific population., (Copyright © 2019 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Abstract NIV adherence ('quantity' of ventilation) has a prognostic impact in amyotrophic lateral sclerosis (ALS). We hypothesized that NIV effectiveness ('quality') could also have a similar impact. NIV effectiveness was evaluated in 82 patients within the first month (M1) and every three months (symptoms, arterial blood bases, and nocturnal pulsed oxygen saturation - SpO2). Kaplan-Meier survival and risk factors for mortality one year after NIV initiation were evaluated. Forty patients were considered 'correctly ventilated' at M1 (Group 1, less than 5% of nocturnal oximetry time with an SpO2<90% - TS90) while 42 were not (Group 2). Both groups were comparable in terms of respiratory and neurological baseline characteristics. Survival was better in Group 1 (75% survival at 12 months) than in Group 2 (43% survival at 12 months, p = 0.002). In 12 Group 2 patients corrective measures were efficient in correcting TS90 at six months. In this subgroup, one-year mortality was not different from that in Group 1. Multivariate analysis identified independent mortality risk factors expectedly including bulbar involvement (HR = 4.31 (1.73 - 10.76), p = 0.002), 'rapid respiratory decline' (HR = 3.55 (1.29 - 9.75), p = 0.014) and vital capacity (HR = 0.97 (0.95 - 0.99), p = 0.010), but also inadequate ventilation in the first month (HR = 2.32 (1.09 - 4.94), p = 0.029). In conclusion, in ALS patients NIV effectiveness to correct nocturnal desaturations is an independent prognostic factor.