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Objective: To assess the diagnostic and surveillance practices of Canadian pediatric subspecialists for children with congenital central hypoventilation syndrome (CCHS)., Methods: The present analysis was a prospective cross-sectional study. A web-based survey was sent to 303 pediatric subspecialists in Canada: 85 pediatric respirologists, 77 pediatric neurologists and 141 neonatologists. The survey included 36 questions about the current diagnostic and surveillance management of pediatric CCHS. Differences in responses among respirologists, neurologists and neonatologists were evaluated for each question, where feasible, and responses were compared with the 2010 American Thoracic Society (ATS) Clinical Policy Statement for CCHS., Results: A total of 83 (27%) participants responded to the survey; the highest survey response rate (40%) was from respirologists. For the diagnosis of CCHS, 25% of respondents did not order genetic testing, either alone or with another test, to make a diagnosis of CCHS. The criteria and tests recommended by the ATS to make a diagnosis of CCHS - genetic testing, diagnosis of exclusion, polysomnogram and plus or minus a hypercapnic challenge - were ordered by 23 (43%) of the 54 respondents. Although polysomnograms were ordered for more than 90% of children with CCHS, only 37% of respirologists aimed for a carbon dioxide range of 35 mmHg to 40 mmHg during polysomnogram titrations., Conclusions: The results demonstrate variability in the diagnostic and surveillance practices of pediatric subspecialists in children with CCHS across Canada. The present study provides an initial needs assessment and demonstrated that there are significant deviations in practice from the 2010 ATS guidelines.