Neuromuscular disorders (NMDs) are common causes of both acute and chronic respiratory failure seen in the Intensive Care Unit. While the pathophysiology of these pathologies is diverse, they result in a final common pathway of muscle weakness, affecting the “ventilatory pump,” leading to a restrictive lung disease pattern. Alongside this primary pathophysiologic effect, NMDs are also known to influence upper airway muscles, impair secretion clearance, and are commonly associated with central component of respiratory dysfunction. Since it was used as a common modality of respiratory support during the poliomyelitis pandemic more than a hundred years ago, Negative Pressure Ventilation (NPV) has been employed in the management of respiratory failure secondary to neuromuscular disorders. Current literature presents both direct and indirect evidence supporting the efficacy of NPV in NMDs. This chapter provides a concise overview of pathophysiology of respiratory failure in NMDs, negative pressure ventilation, possible benefits of NPV in the management of respiratory failure secondary to NMDs, and the supporting evidence of NPV use in this patients’ population.

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Biphasic Cuirass Non-Invasive Ventilation: Neuromuscular/Restrictive Pulmonary Disorders

  • Mohammad Sallam,
  • Maya Rauth,
  • James Baxter,
  • Omar Alibrahim

摘要

Neuromuscular disorders (NMDs) are common causes of both acute and chronic respiratory failure seen in the Intensive Care Unit. While the pathophysiology of these pathologies is diverse, they result in a final common pathway of muscle weakness, affecting the “ventilatory pump,” leading to a restrictive lung disease pattern. Alongside this primary pathophysiologic effect, NMDs are also known to influence upper airway muscles, impair secretion clearance, and are commonly associated with central component of respiratory dysfunction. Since it was used as a common modality of respiratory support during the poliomyelitis pandemic more than a hundred years ago, Negative Pressure Ventilation (NPV) has been employed in the management of respiratory failure secondary to neuromuscular disorders. Current literature presents both direct and indirect evidence supporting the efficacy of NPV in NMDs. This chapter provides a concise overview of pathophysiology of respiratory failure in NMDs, negative pressure ventilation, possible benefits of NPV in the management of respiratory failure secondary to NMDs, and the supporting evidence of NPV use in this patients’ population.