{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T02:14:44Z","timestamp":1740104084516,"version":"3.37.3"},"reference-count":24,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2022,5,5]],"date-time":"2022-05-05T00:00:00Z","timestamp":1651708800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Patients with congenital central hypoventilation syndrome (CCHS) can develop hypoxemia and hypercapnia during exercise. However, there is limited literature on cardiorespiratory responses during submaximal exercise and their correlation with paired\u2010like homeobox 2B (<jats:italic>PHOX2B<\/jats:italic>) genotype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To assess oxygen saturation (SpO<jats:sub>2<\/jats:sub>), end\u2010tidal carbon dioxide (ETCO<jats:sub>2<\/jats:sub>), heart rate (HR), and 6\u2010min\u00a0walk distance (6MWD) during a 6\u2010min\u00a0walk test (6MWT) in CCHS subjects and to correlate them with <jats:italic>PHOX2B<\/jats:italic> genotypes and assisted ventilation (AV) via tracheostomy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this cross\u2010sectional study, subjects with CCHS performed 6MWT with continuous pulse oximetry, HR, and capnography recorded before and during the 6MWT. Medical records were reviewed for <jats:italic>PHOX2B<\/jats:italic> genotype and phenotype data. Patients were categorized based on <jats:italic>PHOX2B<\/jats:italic> genotype and AV via tracheostomy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifteen subjects aged 10.5 (interquartile range 7.9\u221216.2) years completed the 6MWT. Nine subjects used AV via tracheostomy. Seven (47%) subjects developed hypoxemia (SpO<jats:sub>2<\/jats:sub>\u2009\u2264\u200990%, <jats:italic>n<\/jats:italic>\u2009=\u20097) and hypoventilation (ETCO<jats:sub>2<\/jats:sub>\u2009\u2265\u200950\u2009mmHg, <jats:italic>n<\/jats:italic>\u2009=\u20093) during the 6MWT. There was a significant decline from baseline SpO<jats:sub>2<\/jats:sub>, increase from baseline ETCO<jats:sub>2<\/jats:sub>, and increase in HR during the 6MWT (all <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). Subjects had decreased median percent predicted 6MWD (59.7% [50.6%\u221262.5%]). Nadir SpO<jats:sub>2<\/jats:sub> (<jats:italic>p<\/jats:italic>\u2009=\u20090.029) and peak ETCO<jats:sub>2<\/jats:sub> (<jats:italic>p<\/jats:italic>\u2009=\u20090.046) differed significantly between <jats:italic>PHOX2B<\/jats:italic> genotype groups but 6MWD did not (<jats:italic>p<\/jats:italic>\u2009=\u20090.8).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Despite normal oxygenation and ventilation at rest and during sleep on AV, patients with CCHS can develop hypoxemia and hypercapnia during submaximal exercise. Our study highlights the importance of assessing ventilatory responses during submaximal exercise in patients with CCHS regardless of their <jats:italic>PHOX2B<\/jats:italic> genotype.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.25940","type":"journal-article","created":{"date-parts":[[2022,4,23]],"date-time":"2022-04-23T06:49:45Z","timestamp":1650696585000},"page":"1660-1667","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Impaired ventilation during 6\u2010min\u00a0walk test in congenital central hypoventilation syndrome"],"prefix":"10.1002","volume":"57","author":[{"given":"Radhika N.","family":"Ghosh","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonology, Children's Healthcare of Atlanta Emory University Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8145-0430","authenticated-orcid":false,"given":"Lokesh","family":"Guglani","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonology, Children's Healthcare of Atlanta Emory University Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adrianna L.","family":"Westbrook","sequence":"additional","affiliation":[{"name":"Pediatric Biostatistics Core, Department of Pediatrics, Children's Healthcare of Atlanta Emory University Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chad Y.","family":"Mao","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Cardiology, Children's Healthcare of Atlanta Emory University Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shasha","family":"Bai","sequence":"additional","affiliation":[{"name":"Pediatric Biostatistics Core, Department of Pediatrics, Children's Healthcare of Atlanta Emory University Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas G.","family":"Keens","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles University of Southern California Los Angeles California USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0435-2807","authenticated-orcid":false,"given":"Ajay S.","family":"Kasi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonology, Children's Healthcare of Atlanta Emory University Atlanta Georgia 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