title: Respiratory outcome in children with scimitar syndrome.
abstract: OBJECTIVE
  To evaluate respiratory morbidities and lung function tests in the cohort of patients with scimitar syndrome evaluated at our center since 1976.
  
  
  METHODS
  Eighty-one children were investigated. Twenty-six patients died, all with the infantile form. The median duration of follow-up of surviving children was 7.2 years.
  
  
  RESULTS
  A high rate of respiratory morbidities was measured, with 38% and 43% of children reporting pulmonary infections or wheezing episodes during the last 12 months of follow-up, respectively. One-third of children have been rehospitalized for a respiratory cause. Lung function tests were obtained in 20 children. The median value of total lung capacity was 73.0% of the predicted value (IQR, 65.3-86.8), and the median value of the ratio of the forced expiratory volume in one second to the forced vital capacity was -1.26 Z score (-2.25; -0.31). Significantly lower total lung capacity values were obtained in children with the infantile form (P < .005) or with a history of thoracic surgery (P = .002). The ratio of the forced expiratory volume in one second to the forced vital capacity Z score values were significantly lower in boys (P < .05) and in children with a history of wheezing (P = .01). Wheezing episodes were not associated with significant salbutamol-induced reversibility.
  
  
  CONCLUSIONS
  Respiratory complications frequently are observed in children with scimitar syndrome. Pulmonary hypoplasia appears as an independent marker of long-term severity in these patients.
authors.affiliation: Pediatric Pneumology, Necker, AP-HP, Paris, France.
authors.forename: Aude
authors.initials: A
authors.lastname: Chemin
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
authors.forename: Muriel
authors.initials: M
authors.lastname: Le Bourgeois
authors.forename: Marilyne
authors.initials: M
authors.lastname: Levy
authors.forename: Christophe
authors.initials: C
authors.lastname: Delacourt
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D011446:Prospective Studies; D012120:Respiration Disorders / Q000209:etiology*; D012129:Respiratory Function Tests; D012587:Scimitar Syndrome / Q000150:complications* / Q000503:physiopathology*