A) Ribcage (top), abdominal (middle) and chest wall (bottom) volume variations during spontaneous breathing in supine position in a 17-year-old DMD patient.

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a) Ribcage (top), abdominal (middle) and chest wall (bottom) volume variations during spontaneous breathing in supine position in a 17-year-old DMD patient. a) Ribcage (top), abdominal (middle) and chest wall (bottom) volume variations during spontaneous breathing in supine position in a 17-year-old DMD patient. While tidal volume is constant, the thoracoabdominal pattern alternatively changes by passing from abdominal (A, blue lines) to thoracic (B, grey lines) predominance. b) “Loops” (i.e. volume changes of the abdomen versus the ribcage) in representative breaths when either the abdomen (A) or the ribcage (B) are prevalent. The breath of a healthy peer (control) is also reported. The arrows indicate the direction of the loops, i.e. the relative action of inspiratory ribcage muscles and the diaphragm [31]. In healthy subjects, both ribcage and abdomen expand during inspiration, with the latter being predominant. This indicates that in the supine position, the diaphragm is the leading muscle of inspiration, with a reduced contribution of ribcage muscles that mainly act to avoid chest wall distortion. The DMD patient therefore alternates periods of breathing in which inspiration is led by the diaphragm (A) and periods in which ribcage muscles are the leading inspiratory muscles (B). These thoracoabdominal “alternans” are thought to be a strategy adopted to cope with diaphragmatic weakness and fatigue. Note that, however, when the diaphragm leads inspiration (A), its action is reduced compared to the healthy subject (i.e. reduced abdominal expansion) and this is an index of diaphragmatic weakness. Antonella Lo Mauro, and Andrea Aliverti Breathe 2016;12:318-327 ©2016 by European Respiratory Society