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. 2021;100(8):816-825.
doi: 10.1159/000515453. Epub 2021 May 7.

Noninvasive Home Mechanical Ventilation in Adult Myotonic Dystrophy Type 1: A Systematic Review

Bettine A H Vosse  1 , Charlotte Seijger  2 , Nicolle Cobben  1 , Baziel van Engelen  3 , Sander M J van Kuijk  4 , Catharina Faber  5 , Peter Wijkstra  2
Affiliations

Noninvasive Home Mechanical Ventilation in Adult Myotonic Dystrophy Type 1: A Systematic Review

Bettine A H Vosse et al. Respiration. 2021.

Abstract

Introduction: Chronic hypercapnic respiratory failure induces considerable morbidity and mortality in patients with myotonic dystrophy type 1 (DM1). This study systematically reviews the effects of noninvasive home mechanical ventilation (HMV) on gas exchange, quality of life, survival, and compliance in DM1 patients.

Methods: A systematic Medline and Embase search was performed (January 1995 to January 2020). Records were screened for eligibility criteria, data were extracted from included studies, and risk of bias was assessed. We present findings mainly using a narrative synthesis.

Results: Twenty-eight relevant full-text articles were screened for eligibility criteria. Nine studies were included. Randomized controlled trials were not found. Studies had either an observational (n = 8) or interventional (n = 1) design. In the pooled data analysis, HMV showed to improve mean oxygen saturation with 4.8% and decreased mean carbon dioxide values with 3 mm Hg. Compliance varied widely between studies, from no use to more than 12 h per day. Quality of life was not studied extensively, but some studies reported positive effects of HMV on symptoms of chronic respiratory failure. HMV may improve survival in DM1 patients with chronic hypercapnic respiratory failure.

Conclusion: This review shows that HMV can improve gas exchange and relieve symptoms with a possible survival benefit in DM1 patients with chronic hypercapnic respiratory failure. Future studies should focus on developing strategies to optimize the timing of HMV initiation and to promote compliance.

Keywords: Artificial; Mechanical; Myotonic dystrophy; Noninvasive ventilation; Respiration; Respiratory insufficiency; Ventilators.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
PRISMA flow chart illustrating the study selection process. PRISMA, Preferred Reporting Items for Systematic Reviews and Meta-Analyses.
Fig. 2
Fig. 2
Forest plots: pooled data on gas exchange. Change in PCO2 after 1 month on HMV (a); change in PO2 after 1 month on HMV (b); change in SaO2 after 1 month on HMV (c). PCO2, partial pressure of carbon dioxide in blood; PO2, partial pressure of oxygen in blood; SaO2, oxygen saturation; HMV, home mechanical ventilation.
See this image and copyright information in PMC

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