Effect and impact of mechanical ventilation in myotonic dystrophy type 1: a prospective cohort study. Issue 11 (23rd March 2018)

• Record Type: Journal Article
• Authors: Boussaïd, Ghilas; Prigent, Hélène; Laforet, Pascal; Raphaël, Jean-Claude; Annane, Djillali; Orlikowski, David; Lofaso, Frédéric
• Is Part Of: Thorax. Volume 73:Issue 11(2018)
• Page Start: 1075
• Page End: 1078
• Publication Date: 2018-03-23
• Subjects: respiratory muscles -- assisted ventilation -- non-invasive ventilation -- clinical epidemiology; Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thoraxjnl-2017-210610
• Languages: English
• ISSNs: 0040-6376

Abstract

Abstract : Few studies have assessed the impact of home ventilation in patients with myotonic dystrophy type 1 (DM1) and no specific recommendations are available. We assessed the survival associated with category of home ventilation adherence of patients with DM1 followed up at a home ventilation unit using a Cox proportional hazards model. 218 patients were included; those who refused or delayed their acceptance of non-invasive ventilation were at higher risk for severe events (invasive ventilation or death) (P=0.03). Risk of death was associated with orthopnoea (HR 2.37; 95% CI 1.17 to 4.80; P<0.02) and adherence category (100 to 90% vs >75%: HR 3.26; 95% CI 1.32 to 8.04; P<0.03). Failure to use home ventilation as prescribed may be associated with increased mortality in patients with DM1.