title: Intelligent volume-assured pressured support (iVAPS) for the treatment of congenital central hypoventilation syndrome.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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(tcCO2) 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.
authors.forename: Abdullah
authors.initials: A
authors.lastname: Khayat
authors.affiliation: Children's Hospital of Wisconsin, Milwaukee, WI, USA.
authors.forename: Debra
authors.initials: D
authors.lastname: Medin
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.
authors.forename: Faiza
authors.initials: F
authors.lastname: Syed
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.|University of Toronto, Toronto, ON, Canada.
authors.forename: Theo J
authors.initials: TJ
authors.lastname: Moraes
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.
authors.forename: Saadoun
authors.initials: S
authors.lastname: Bin-Hasan
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.|University of Toronto, Toronto, ON, Canada.
authors.forename: Indra
authors.initials: I
authors.lastname: Narang
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.|University of Toronto, Toronto, ON, Canada.
authors.forename: Suhail
authors.initials: S
authors.lastname: Al-Saleh
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada. reshma.amin@sickkids.ca.|University of Toronto, Toronto, ON, Canada. reshma.amin@sickkids.ca.
authors.forename: Reshma
authors.initials: R
authors.lastname: Amin
mesh_terms: D002648:Child; D002675:Child, Preschool; D045422:Continuous Positive Airway Pressure / Q000295:instrumentation*; D005260:Female; D006801:Humans; D007040:Hypoventilation / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D007223:Infant; D008297:Male; D017286:Polysomnography; D012189:Retrospective Studies; D020182:Sleep Apnea, Central / Q000175:diagnosis / Q000628:therapy*; D013813:Therapy, Computer-Assisted / Q000295:instrumentation*; D016896:Treatment Outcome
keywords: Central sleep apnea; Congenital central hypoventilation syndrome; Intelligent volume-assured pressure support; Pediatrics; Sleep disordered breathing