Abstract
Background:
The clinical course of COVID-19 in patients with congenital central hypoventilation syndrome (CCHS) is unknown.
Methods:
We conducted a cross-sectional questionnaire study in 43 patients with CCHS who had COVID-19.
Results:
The median age of patients was 11 [interquartile range (IQR) 6–22] years and 53.5% required assisted ventilation (AV) through tracheostomy. Disease severity ranged from asymptomatic infection (12%) to severe illness with hypoxemia (33%) and hypercapnia requiring emergency care/hospitalization (21%), increased AV duration (42%), increased ventilator settings (12%), and supplemental oxygen demand (28%). The median duration to return to baseline AV (n = 20) was 7 (IQR 3–10) days. Patients with polyalanine repeat mutations required increased AV duration compared with those with nonpolyalanine repeat mutations (P = 0.048). Patients with tracheostomy required increased oxygen during illness (P = 0.02). Patients aged ≥18 years took longer to return to baseline AV (P = 0.04).
Conclusions:
Our study suggests that all patients with CCHS should be vigilantly monitored during COVID-19 illness.
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