Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, Paris, France.
Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, Paris, France Département "R3S", Service des Pathologies du Sommeil AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
J Neurol Neurosurg Psychiatry. 2016 Oct;87(10):1045-50. doi: 10.1136/jnnp-2015-312606. Epub 2016 Apr 18.
Non-invasive ventilation (NIV) is part of standard care in amyotrophic lateral sclerosis (ALS). Intolerance or unavailability of NIV, as well as the quality of correction of nocturnal hypoventilation, has a direct impact on prognosis.
We describe the importance of NIV failure due to upper airway obstructive events, the clinical characteristics, as well as their impact on the prognosis of ALS.
Retrospective analysis of the data of 190 patients with ALS and NIV in a single centre for the period 2011-2014. 179 patients tolerating NIV for more than 4 h per night without leaks were analysed.
Among the 179 patients, after correction of leaks, 73 remained inadequately ventilated at night (defined as more than 5% of the night spent at <90% of SpO2), as a result of obstructive events in 67% of cases (n=48). Patients who remained inadequately ventilated after optimal adjustment of ventilator settings presented with shorter survival than adequately ventilated patients. Unexpectedly, patients with upper airway obstructive events without nocturnal desaturation and in whom no adjustment of treatment was therefore performed also presented with shorter survival. On initiation of NIV, no difference was demonstrated between patients with and without upper airway obstructive events. In all patients, upper airway obstruction was concomitant with reduction of ventilatory drive.
This study shows that upper airway obstruction during NIV occurs in patients with ALS and is associated with poorer prognosis. Such events should be identified as they can be corrected by adjusting ventilator settings.
在肌萎缩侧索硬化症（ALS）中，无创通气（NIV）是标准治疗的一部分。NIV 不耐受或不可用，以及夜间低通气纠正的质量，直接影响预后。
我们描述了由于上气道阻塞事件导致的 NIV 失败的重要性，以及其临床特征及其对 ALS 预后的影响。
回顾性分析了 2011-2014 年期间在单一中心接受 NIV 的 190 例 ALS 患者的数据。分析了 179 例夜间 NIV 耐受时间超过 4 小时且无泄漏的患者。
在 179 例患者中，在纠正泄漏后，由于阻塞事件，仍有 73 例患者夜间通气不足（定义为超过 5%的时间 SpO2<90%），在 67%的情况下（n=48）。在最佳调整呼吸机设置后仍通气不足的患者比通气充足的患者存活时间更短。出乎意料的是，在上气道阻塞但无夜间低氧血症且未调整治疗的患者中，生存时间也更短。在开始使用 NIV 时，有上气道阻塞事件的患者与没有上气道阻塞事件的患者之间没有差异。在所有患者中，上气道阻塞与通气驱动的降低同时发生。
本研究表明，在 ALS 患者中，NIV 期间上气道阻塞很常见，且与预后不良有关。应识别这些事件，因为可以通过调整呼吸机设置来纠正。
