Central CO2 chemosensitivity and CO2 controller gain independently contribute to daytime Pco2 in young subjects with congenital central hypoventilation syndrome

Abstract

Altered central CO2 chemosensitivity is a hallmark of congenital central hypoventilation syndrome (CCHS). Peripheral CO2 chemosensitivity can be partly assessed by controller gain measurement obtained from tidal breathing recording. In young subjects with CCHS, this study shows that both central and peripheral CO2 sensitivities independently contribute to daytime Pco2. Hypocapnia during nighttime-assisted ventilation is associated with higher peripheral chemosensitivity that is further associated with lesser arterial desaturation at walk.