Department of Pulmonology, University Hospital of S. João, Faculty of Medicine, University of Porto, Av. Prof. Hernani Monteiro, Porto, Portugal.
Department of Physical Medicine and Rehabilitation, Rutgers University - New Jersey Medical School, USA.
Pulmonology. 2021 Nov-Dec;27(6):509-517. doi: 10.1016/j.pulmoe.2021.06.007. Epub 2021 Oct 14.
Typically, patients with progressive neuromuscular disorders (NMDs) develop acute respiratory failure (ARF), are intubated, and when failing spontaneous breathing trials (SBTs) undergo a tracheotomy and receive tracheostomy mechanical ventilation (TMV). However, increasing numbers of patients use nasal noninvasive ventilation (NIV), initially for sleep and this is extended to continuous dependence (CNVS). This can be used as a strategy to assist in successful extubation . We retrospectively reviewed 19 centers offering CNVS and mechanical insufflation-exsufflation (MI-E) as an alternative to TMV.
Centers with publications or presentations concerning CNVS outcomes data were pooled for amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy type 1 (SMA1). Progression to CNVS dependence without hospitalization, duration of dependence, and extubations and decannulations to CNVS were recorded. Prolongation of life was defined by duration of CNVS dependence without ventilator free breathing ability (VFBA).
There were 1623 part time (<23 h/day) NVS users with ALS, DMD, and SMA1 from 19 centers in 16 countries of whom 761 (47%) were CNVS dependent for 2218 patient-years. This included: 335 ALS patients for a mean 1.2 ± 1.0 (range to 8) years each; 385 DMD patients for 5.4 ± 1.6 (range to 29) years; and 41 SMA1 patients for 5.9 ± 1.8 (range to 20) years. Thirty-five DMD and ALS TMV users were decannulated to CNVS and MI-E. At data collection 494 (65%) patients were CNVS dependent but 110 (74 of whom with bulbar ALS), had undergone tracheotomies.
ALS, DMD, and SMA1 patients can become CNVS dependent without requiring hospitalization but CNVS cannot be used indefinitely for many patients with advanced upper motor neuron diseases.
通常，患有进行性神经肌肉疾病（NMD）的患者会出现急性呼吸衰竭（ARF），需要插管，如果在自主呼吸试验（SBT）中失败，会进行气管切开和接受气管造口机械通气（TMV）。然而，越来越多的患者使用鼻内无创通气（NIV），最初是用于睡眠，并且这种方法会扩展到持续依赖（CNVS）。这可以作为一种策略来协助成功拔管。我们回顾了 19 个提供 CNVS 和机械通气-呼气（MI-E）的中心，将其作为 TMV 的替代方法。
将有 CNVS 结果数据的出版物或报告的中心汇集在一起，用于肌萎缩侧索硬化症（ALS）、杜氏肌营养不良症（DMD）和脊髓性肌萎缩症 1 型（SMA1）。记录无住院的 CNVS 依赖进展、依赖持续时间以及拔管和向 CNVS 过渡。CNVS 无呼吸机自由呼吸能力（VFBA）的依赖持续时间的延长被定义为寿命的延长。
在来自 16 个国家的 19 个中心的 1623 名部分时间（<23 小时/天）NVS 用户中，有 335 名 ALS 患者、385 名 DMD 患者和 41 名 SMA1 患者，平均分别依赖 CNVS 1.2±1.0 年（范围为 8 年）、5.4±1.6 年（范围为 29 年）和 5.9±1.8 年（范围为 20 年）。35 名 DMD 和 ALS TMV 用户被气管切开并使用 CNVS 和 MI-E。在数据收集时，494 名（65%）患者依赖 CNVS，但 110 名（其中 74 名患有延髓 ALS）已进行气管切开。
ALS、DMD 和 SMA1 患者可以在无需住院的情况下成为 CNVS 依赖者，但许多患有晚期上运动神经元疾病的患者不能无限期地使用 CNVS。
