title: Relationship between obstructive sleep apnea cardiac complications and sleepiness in children with Down syndrome.
abstract: OBJECTIVE
  Children with Down syndrome (DS) have a high rate of pulmonary hypertension and sleepiness. They also have a high prevalence of obstructive sleep apnea syndrome (OSAS). We hypothesized that OSAS was associated with cardiovascular dysfunction and sleepiness in children with DS, and that this dysfunction was partly reversible.
  
  
  METHODS
  A total of 23 children with DS, aged 8-19 years, were evaluated with polysomnography, echocardiography, and measurement of brain natriuretic peptide (BNP). Children having OSAS were randomized to four months of actual or sham continuous positive airway pressure (CPAP) in a double-blinded fashion.
  
  
  RESULTS
  Of the total participants, 20 (87%) had OSAS. On echocardiography, no participant was found to have pulmonary hypertension, and all participants had a BNP <10 pg/mL. The early/tissue Doppler (E/e') of the lateral mitral annulus, a measure of worse left ventricular (LV) diastolic function, correlated with the arousal index (r = 0.42, p = 0.043) and apnea hypopnea index (AHI; r = 0.61, p = 0.002) and inversely with the SpO2 nadir (r = -0.61, p = 0.002). Participants with OSAS had a high pediatric Epworth score [median interquartile range (IQR) = 8(4,9)],correlating with the arousal index (r = 0.49, p = 0.016). At four months, there were no changes in cardiovascular outcomes or sleepiness between those on actual versus sham CPAP. Hours of actual CPAP use was associated with improved E/e' mitral lateral (r = -0.48, p = 0.044), but surprisingly also correlated with LV mass z-score (r = 0.54, p = 0.018).
  
  
  CONCLUSIONS
  In children with DS, LV diastolic function correlated with OSAS severity, with improvement with CPAP use. There was a tendency towards increased sleepiness in those with OSAS, which correlated with the arousal index. Larger studies are warranted to confirm these findings.
authors: Sleep Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Sofia
authors: S
authors: Konstantinopoulou
authors: Sleep Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Ignacio E
authors: IE
authors: Tapia
authors: Clinical and Translational Research Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Ji Young
authors: JY
authors: Kim
authors: Sleep Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Melissa S
authors: MS
authors: Xanthopoulos
authors: Clinical and Translational Research Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Jerilynn
authors: J
authors: Radcliffe
authors: Cardiac Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Meryl S
authors: MS
authors: Cohen
authors: Cardiac Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Brian D
authors: BD
authors: Hanna
authors: Trisomy 21 Program, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Mary
authors: M
authors: Pipan
authors: Sleep Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Christopher
authors: C
authors: Cielo
authors: Clinical and Translational Research Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Allison J
authors: AJ
authors: Thomas
authors: Division of Gastroenterology, Nutrition, and Hepatology, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Babette
authors: B
authors: Zemel
authors: Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors: Raouf
authors: R
authors: Amin
authors: Sleep Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Ruth
authors: R
authors: Bradford
authors: Sleep Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Joel
authors: J
authors: Traylor
authors: Clinical and Translational Research Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors: Justine
authors: J
authors: Shults
authors: Sleep Center, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, USA. Electronic address: marcus@email.chop.edu.
authors: Carole L
authors: CL
authors: Marcus
mesh_terms: D000293:Adolescent; D002318:Cardiovascular Diseases / Q000209:etiology*; D002648:Child; D045422:Continuous Positive Airway Pressure; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D017286:Polysomnography; D020181:Sleep Apnea, Obstructive / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012893:Sleep Wake Disorders / Q000209:etiology*; D055815:Young Adult
keywords: Down syndrome; Noninvasive ventilation; Sleep-disordered breathing