Mouthpiece ventilation in neuromuscular disorders: Narrative review of technical issues important for clinical success

Respir Med. 2021 Apr-May;180:106373. doi: 10.1016/j.rmed.2021.106373. Epub 2021 Mar 24.


In neuromuscular disorders (NMDs), nocturnal non-invasive ventilation (NIV) via a nasal mask is offered when hypercapnic respiratory failure occurs. With disease progression, nocturnal NIV needs to be extended into the daytime. Mouthpiece ventilation (MPV) is an option for daytime NIV. MPV represents a difficult task for home ventilators due to rapidly changing load conditions resulting from intermittent connections and disconnections from MPV circuit. The 252nd ENMC International Expert Workshop, held March 6th to 8th 2020 in Amsterdam, reported general guidelines for management of daytime MPV in NMDs. This report could not present all the detail regarding the technical issues important for clinical success of MPV. Based on the expert workshop discussions and the evidence from existing studies, the current narrative review aims to identify the technical issues of MPV and offers guidance via a decisional algorithm and educational figures providing relevant information that is important for successful implementation of MPV.

Keywords: MPV; Mouthpiece ventilation; NMD; Neuromuscular disorders; Non-invasive ventilation.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Education
  • Humans
  • Neuromuscular Diseases / complications*
  • Noninvasive Ventilation / methods*
  • Patient Education as Topic
  • Practice Guidelines as Topic
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / prevention & control*
  • Respiratory Insufficiency / therapy