Intelligent volume-assured pressured support (iVAPS) for the treatment of congenital central hypoventilation syndrome

Congenital central hypoventilation syndrome (CCHS) is characterized by ventilatory insensitivity to hypercapnia and hypoxemia during sleep and/or wakefulness. Management of CCHS includes a long-term ventilation. However, ventilation can be challenging given differences in the control of breathing during different sleep stages. Intelligent volume-assured pressure support (iVAPS) is a mode of Bi-level positive airway pressure (BPAP) ventilation in which the pressure support is modulated to ensure a constant alveolar ventilation. The aim of this study was to determine if BPAP with iVAPS mode is more effective at controlling hypercapnia than BPAP with spontaneous/timed (S/T) mode. A retrospective chart review of CCHS patients who underwent both a titration polysomnogram (PSG) with standard BPAP S/T mode and a consecutive follow-up study with BPAP iVAPS mode at The Hospital for Sick Children, Toronto, Canada, between January 1, 2013 and September 30, 2015 were included. Comparisons were made between S/T mode and iVAPS mode. Eight (four males) children with CCHS were included. The median (IQR) age at the time of PSG using Bi-level ventilation with S/T mode for study participants was 10.0 (IQR 8.4, 11.6) years followed by PSGs with iVAPS mode, median age 10.6 (IQR 9.1, 12.5) years. The non-rapid eye movement (NREM) peak transcutaneous CO2 (tcCO(2)) median (IQR) for iVAPS was 43.0 (40.0-46.0-) mmHg versus 46.5 (45.0-48.0) mmHg for S/T mode, (p value < 0.05). iVAPS was associated with a reduction in the maximum tcCO2 during NREM sleep as compared to traditional S/T mode. Prospective, longitudinal studies are needed to evaluate the benefits of BPAP therapy iVAPS mode for the treatment of pediatric CCHS.