Efficiency of invasive mechanical ventilation during sleep in Duchenne muscular dystrophy

Abstract

Inspiratory unintentional leaks (IULs) during noninvasive ventilation (NIV) adversely affect the sleep and the effectiveness of mechanical ventilation (MV). The aim of this study was to assess the effects of nocturnal IULs in Duchenne muscular dystrophy (DMD) patients with a tracheostomy and uncuffed tube comparatively with NIV patients. Polysomnography with transcutaneous partial pressure of carbon dioxide (PtcCO(2)) recording and blood gas measurement was performed in 26 stable tracheostomized DMD patients using home MV, among whom 11 were matched with NIV patients. IULs occurred during 29.4% [1.7-61.9%] (median [IQR]) of the total sleep time. By univariate regression analysis, the closest correlation with IUL duration was for daytime base excess (r(2)=0.69, P<0.0001), followed by daytime bicarbonate level. In a stepwise multiple regression analysis, only base excess remained significantly correlated. Sleep and respiratory parameters improved in the four patients who agreed to use cuffed tubes. Tracheostomized patients had lower maximal PtcCO(2) (P=0.02) and base excess values (P=0.045) compared to NIV controls. Tracheostomy does not guarantee that MV is effective during sleep, as IULs may occur, but ensures better nocturnal gas exchanges than NIV. DMD patients should be evaluated using at least blood gas measurement, nocturnal oximetry, and PtcCO(2) monitoring.