Effect of Facemask in Congenital Central Hypoventilation Syndrome.

Publication date: Dec 01, 2023

Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder with a mutation in the PHOX2B gene. Patients need ventilatory support by noninvasive ventilation or tracheostomy to treat alveolar hypoventilation. Patients with CCHS have a defect in chemosensitivity signal integration. Recently, due to the COVID-19 pandemic, the entire world has had to get used to wearing medical masks (MM). The aim of the study was to evaluate the effect of an MM on gas exchange and to determine the role of central and peripheral chemoresponsiveness on the partial pressure of transcutaneous carbon dioxide (PtcCO2) in patients with CCHS wearing an MM. This study was based on the analysis of recordings obtained without and with an MM during hospitalization and was conducted to assess the impact of MM on PtcCO2 and SpO2 recordings with the SenTec Digital Monitor and their relationships with peripheral CO2 chemosensitivity obtained during tidal breathing measurement and with the hypercapnic hyperoxic ventilatory response. Sixteen patients were included (13 boys) and were 10. 2 (7. 5; 18. 5) years old. The use of an MM had a negative impact on gas exchange in patients with CCHS. The median PtcCO2 increased significantly. Peripheral chemosensitivity correlated with MM-induced PtcCO2 changes (R = -0. 72, p = 0. 005), but central chemosensitivity (the hypercapnic ventilator response slope) did not (R = -0. 22, p = 0. 510). The use of an MM had a negative impact on gas exchange in patients with CCHS.

Concepts Keywords
Gas Chemosensitivity
Genetic Facemask
Hyperoxic Gas exchange


Type Source Name
disease MESH Congenital Central Hypoventilation Syndrome
disease MESH genetic disorder
disease VO gene
disease MESH hypoventilation
disease MESH COVID-19 pandemic
drug DRUGBANK Carbon dioxide

Original Article

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