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Continuous noninvasive ventilatory support for patients with neuromuscular or chest wall disease

John R Bach, MD
Section Editors
Talmadge E King, Jr, MD
R Sean Morrison, MD
Deputy Editor
Geraldine Finlay, MD


Patients with neuromuscular or chest wall disease develop respiratory muscle dysfunction that can result in difficulty clearing airway secretions with ventilation-perfusion mismatching, and alveolar hypoventilation. Noninvasive ventilatory assistance is usually first needed at night, but eventually, daytime assistance may also be needed until many patients become dependent on continuous noninvasive ventilatory support (CNVS). Indeed, virtually all patients with neuromuscular disease except those with bulbar amyotrophic lateral sclerosis (ALS) that causes stridor and upper airway collapse can avoid tracheostomy indefinitely by using noninvasive inspiratory and expiratory aids [1].

The use of CNVS will be reviewed here. Nocturnal ventilatory assistance/support, the types of ventilators, and the role of tracheostomy are discussed separately (see "Practical aspects of nocturnal noninvasive ventilation in neuromuscular and chest wall disease"). In addition, intubated patients and those dependent on up to continuous tracheostomy mechanical ventilation (CTMV) can be extubated or decannulated to CNVS.


Symptomatic alveolar hypoventilation is the primary factor determining the need for ongoing nocturnal ventilatory assistance. Typical symptoms include fatigue, exertional dyspnea, reduced appetite, inattention, and impaired concentration and memory. Initially, hypoventilation occurs during rapid eye movement (REM) sleep and is manifest by oxyhemoglobin desaturation and hypercapnia. Hypoventilation subsequently extends throughout sleep and eventually into daytime hours [2,3]. (See "The effect of sleep in patients with neuromuscular and chest wall disorders".)

Symptoms (eg, dyspnea, somnolence, fatigue) and blood gas derangements related to chronic hypoventilation are typically relieved by nocturnal noninvasive positive pressure ventilatory assistance/support (NVS; noninvasive ventilatory support). While the effect of limiting the application of NVS to nocturnal-only does not result in markedly prolonged survival [4], clinicians who understand and use up to continuous NVS (CNVS) report decades of prolonged survival for patients with neuromuscular (NMD) and chest wall diseases. When the need for ventilatory support extends into daytime hours and is ultimately needed up to continuously, the properly informed patient can use it indefinitely as an alternative to tracheostomy ventilation.


The respiratory muscles can be aided by manually or mechanically applying forces to the body or delivering intermittent pressure to the airway. Some devices assist inspiratory muscles, whereas others facilitate coughing, predominantly by assisting expiratory muscles. Specific types of useful devices include the following [5,6]:


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Literature review current through: Mar 2017. | This topic last updated: Aug 06, 2015.
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