Impaired ventilation during 6-min walk test in congenital central hypoventilation syndrome.
Radhika N GhoshLokesh GuglaniAdrianna L WestbrookChad Y MaoShasha BaiThomas G KeensAjay S KasiPublished in: Pediatric pulmonology (2022)
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 PHOX2B genotype.