Factors influencing compliance with non-invasive ventilation at long-term in patients with myotonic dystrophy type 1: A prospective cohort

•Compliance with non-invasive ventilation is poor in patients with no subjective symptoms of hypercapnia.•Cessation is more frequent in the cases of excessive leaks, ventilator dysfunction, or high body mass index.•Cessation is less frequent for patients with a professional occupation.

This study evaluated compliance with non-invasive ventilation in patients with myotonic dystrophy type 1 and identified predictors of cessation at 5 years in a cohort of patients followed in a specialist center for Neuromuscular Diseases in France. Mechanical ventilation in these patients poses a very strong challenge to caregivers.Factors predicting relative compliance were identified using multivariate linear regressions. Cox proportional-hazards regression was used to estimate hazard ratios associated with risk of cessation. One hundred and twenty-eight patients were included. Compliance during the first year was higher when symptoms of respiratory failure were initially present (+25%, p < 0.003) and lower in the case of acute respiratory failure (−29%, p < 0.003). Long-term compliance was associated with symptoms of respiratory failure (+52%, p < 0.0001) and nocturnal arterial oxygen desaturation (+23%, p < 0.007). Cessation was more frequent in the cases of excessive leaks (HR = 7.81, IC [1.47-41.88], p < 0.01), ventilator dysfunction requiring emergency technical intervention (HR = 12.58, IC [1.22-129.69], p < 0.03) or high body mass index (p < 0.02). Cessation was less frequent for patients with a professional occupation or undergoing professional training (HR = 0.11, IC [0.02-0.77], p < 0.02). Compliance with non-invasive ventilation is poor in patients with no subjective symptoms of respiratory failure. It may be improved with appropriate education and follow-up.