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

Michel Toussaint; Michelle Chatwin; Miguel R Gonçalves; Jésus Gonzalez-Bermejo; Joshua O Benditt; Doug McKim; Jesus Sancho; Brit Hov; Valeria Sansone; Hélène Prigent; Annalisa Carlucci; Peter Wijkstra; Barbara Garabelli; Joan Escarrabill; Tiago Pinto; Nicolas Audag; Laura Verweij-van den Oudenrijn; Adam Ogna; Wendy Hughes; Christian Devaux; Johann Chaulet; Tiina Andersen; ENMC Respiratory Therapy Consortium

doi:10.1016/j.rmed.2021.106373

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.

Authors

Michel Toussaint¹, Michelle Chatwin², Miguel R Gonçalves³, Jésus Gonzalez-Bermejo⁴, Joshua O Benditt⁵, Doug McKim⁶, Jesus Sancho⁷, Brit Hov⁸, Valeria Sansone⁹, Hélène Prigent¹⁰, Annalisa Carlucci¹¹, Peter Wijkstra¹², Barbara Garabelli¹³, Joan Escarrabill¹⁴, Tiago Pinto¹⁵, Nicolas Audag¹⁶, Laura Verweij-van den Oudenrijn¹⁷, Adam Ogna¹⁸, Wendy Hughes¹⁹, Christian Devaux²⁰, Johann Chaulet²¹, Tiina Andersen²²; ENMC Respiratory Therapy Consortium

Affiliations

¹ Neuromuscular Excellency Centre VUB- Inkendaal, Center for Home Mechanical Ventilation ZH Inkendaal Rehabilitation Hospital, Brussels, Belgium. Electronic address: michel.toussaint@inkendaal.be.
² Clinical and Academic Department of Sleep and Breathing, Royal Brompton, London, UK. Electronic address: M.Chatwin@rbht.nhs.uk.
³ Noninvasive Ventilatory Support Unit, Emergency and Intensive Care Medicine Department, Pulmonology Department, São João University Hospital. Faculty of Medicine, University of Porto, Portugal. Electronic address: goncalvesmr@gmail.com.
⁴ Sorbonne-Université, Service de pneumologie et réanimation respiratoire, Groupe hospitalier de la Pitié-Salpêtrière-Charles Foix, Paris, France. Electronic address: prjesusgonzalez@gmail.com.
⁵ University of Washington Medical Center, Seattle, USA. Electronic address: benditt@u.washington.edu.
⁶ University of Ottawa, CANVent Respiratory Services, The Ottawa Hospital Sleep Centre, Canada. Electronic address: dmckim@toh.ca.
⁷ Respiratory Care Unit, Respiratory Medicine Department, Hospital Clínico Universitario, Health Research Institute INCLIVA, Valencia, Spain. Electronic address: jesus.sancho@uv.es.
⁸ Paediatric Department, Oslo University Hospital, Oslo, Norway. Electronic address: brit.hov@online.no.
⁹ The NEMO Clinical Center, Neurorehabilitation Unit, University of Milan, Italy. Electronic address: valeria.sansone@centrocliniconemo.it.
¹⁰ Service de Physiologie et Explorations Fonctionnelles, Hôpital Raymond Poincaré, GHU PIFO, APHP, Garches, France; UFR Simone Veil, Université de Versailles, Saint Quentin en Yvelines, Montigny le Bretonneux, France. Electronic address: helene.prigent@aphp.fr.
¹¹ Pulmonary Rehabilitation and Weaning Center, Istituti Clinici Scientifici-Maugeri, Pavia, Italy. Electronic address: carlucci.annalisa@libero.it.
¹² Department of Home Mechanical Ventilation and Pulmonary Diseases, University Medical Center Groningen, Groningen, the Netherlands. Electronic address: p.j.wijkstra@umcg.nl.
¹³ Respiratory Unit, Neuromuscular OmniCentre (NeMO), Niguarda Hospital, Milan, Italy. Electronic address: Barbara.garabelli87@gmail.com.
¹⁴ Hospital Clínic-Barcelona & Master Plan For Respiratory Diseases, Ministry of Health (Government of Catalonia), Barcelona, Spain. Electronic address: escarrabill@clinic.cat.
¹⁵ Lung Function and Ventilation Unit - Pulmonology Department, São João University Hospital, Porto, Portugal. Electronic address: tiagoffpinto@gmail.com.
¹⁶ Unité de Pneumologie pédiatrique, Cliniques universitaires Saint-Luc, Brussels, Belgium. Electronic address: nicolas.audag@uclouvain.be.
¹⁷ Wilhelmina Kinderziekenhuis, Divisie Kinderen, Centrum voor Thuisbeademing, Utrecht, the Netherlands. Electronic address: L.Verwey@umcutrecht.nl.
¹⁸ Servizio di pneumologia, Ospedale Regionale di Locarno, Switzerland. Electronic address: adam.ogna@eoc.ch.
¹⁹ Myotubular Trust, UK. Electronic address: wendyhughes@myotubulartrust.org.
²⁰ Département des affaires Médicales, AFM, France. Electronic address: CDEVAUX@afm-telethon.fr.
²¹ France. Electronic address: papou1981@gmail.com.
²² Norwegian Advisory Unit on Home Mechanical Ventilation, Thoracic Department, Haukeland University Hospital, Bergen, Norway. Electronic address: tiina.maarit.andersen@helse-bergen.no.

PMID: 33798870
DOI: 10.1016/j.rmed.2021.106373

Abstract

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