PT - JOURNAL ARTICLE AU - Taytard, Jessica AU - Niérat, Marie-Cécile AU - Gand, Camille AU - Lavault, Sophie AU - Morélot-Panzini, Capucine AU - Patout, Maxime AU - Serresse, Laure AU - Wattiez, Nicolas AU - Bodineau, Laurence AU - Straus, Christian AU - Similowski, Thomas TI - Short-term cognitive loading deteriorates breathing pattern and gas exchange in adult patients with congenital central hypoventilation syndrome AID - 10.1183/23120541.00408-2022 DP - 2022 Jan 01 TA - ERJ Open Research PG - 00408-2022 4099 - http://openres.ersjournals.com/content/early/2022/10/06/23120541.00408-2022.short 4100 - http://openres.ersjournals.com/content/early/2022/10/06/23120541.00408-2022.full AB - Question Human PHOX2B mutations result in life-threatening sleep-related hypoventilation (congenital central hypoventilation syndrome, CCHS). Most patients retain ventilatory activity when awake through a respiratory-related cortical network. We hypothesised that this need to mobilise cortical resources to breathe would lead to breathing-cognition interferences during cognitive loading.Patients and Methods Seven adult CCHS patients (5 women; median age 21) performed standard neuropsychological tests (Paced Auditory Serial Addition Test –calculation capacity, working memory, sustained and divided attention–; Trail Making Test –visuospatial exploration capacity, cognitive processing speed, attentional flexibility–; Corsi block-tapping test –visuospatial memory, short-term memory, working memory–), during unassisted breathing and under ventilatory support. Ventilatory variables and transcutaneous haemoglobin oxygen saturation were recorded. Cortical connectivity changes between unassisted breathing and ventilatory support were assessed using electroencephalographic recordings (EEG).Results Baseline performances were lower than expected in individuals this age. During unassisted breathing, cognitive loading coincided with increased breathing variability and decreases in oxygen saturation inversely correlated with an increasing number of apnoeic cycles/minute (rho −0.46, 95% confidence interval −0.76 to −0.06, p=0.01). During ventilatory support, cognitive tasks did not disrupt breathing pattern and were not associated with decreased oxygen saturation. Ventilatory support was associated with changes in EEG cortical connectivity but not with improved test performances.Conclusions Acute cognitive loads induce oxygen desaturation in adult CCHS patients during unassisted breathing, but not under ventilatory support. This justifies considering the use of ventilatory support during mental tasks in CCHS patients, to avoid repeated episodes of hypoxia.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Jessica TaytardConflict of interest: Marie-Cécile NiératConflict of interest: Camille GandConflict of interest: Sophie LavaultConflict of interest: Capucine Morélot-PanziniConflict of interest: Maxime PatoutConflict of interest: Laure SerresseConflict of interest: Nicolas WattiezConflict of interest: Laurence BodineauConflict of interest: Christian StrausConflict of interest: Thomas Similowski