title: Airway ciliary dysfunction and sinopulmonary symptoms in patients with congenital heart disease.
abstract: BACKGROUND
  Patients with congenital heart disease with heterotaxy exhibit a high prevalence of abnormal airway ciliary motion and low nasal nitric oxide, characteristics associated with primary ciliary dyskinesia, a reflection of the role of motile cilia in airway clearance and left-right patterning.
  
  
  OBJECTIVE
  To assess the potential broader clinical significance of airway ciliary dysfunction in congenital heart disease, we assessed the prevalence of ciliary dysfunction versus respiratory symptoms in patients with congenital heart disease with or without heterotaxy.
  
  
  METHODS
  Patients with a broad spectrum of congenital heart disease were recruited (n = 218), 39 with heterotaxy. Nasal nitric oxide measurements and nasal biopsies for ciliary motion video microscopy were conducted. Sinopulmonary symptoms were reviewed by questionnaire.
  
  
  RESULTS
  A high prevalence of ciliary motion defects (51.8%) and low or borderline low nasal nitric oxide levels (35.5%) were observed in patients with congenital heart disease with or without heterotaxy. Patients with ciliary motion defects or low nasal nitric oxide showed increased sinopulmonary symptoms, with most respiratory symptoms seen in those with both abnormal ciliary motion and low nitric oxide. Multivariate analysis showed that abnormal ciliary motion and low nasal nitric oxide were more important in determining risk of sinopulmonary symptoms than heterotaxy status.
  
  
  CONCLUSIONS
  Patients with congenital heart disease without heterotaxy exhibit a high prevalence of abnormal ciliary motion and low nasal nitric oxide. This was associated with more sinopulmonary symptoms. These findings suggest that patients with a broad spectrum of congenital heart disease and respiratory symptoms may benefit from screening for ciliary dysfunction and implementation of medical interventions to reduce sinopulmonary morbidities.
authors.affiliation: 1 Department of Pediatrics, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center (UPMC), and.
authors.forename: Andrea S
authors.initials: AS
authors.lastname: Garrod
authors.forename: Maliha
authors.initials: M
authors.lastname: Zahid
authors.forename: Xin
authors.initials: X
authors.lastname: Tian
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Francis
authors.forename: Omar
authors.initials: O
authors.lastname: Khalifa
authors.forename: William
authors.initials: W
authors.lastname: Devine
authors.forename: George C
authors.initials: GC
authors.lastname: Gabriel
authors.forename: Linda
authors.initials: L
authors.lastname: Leatherbury
authors.forename: Cecilia W
authors.initials: CW
authors.lastname: Lo
mesh_terms: D000293:Adolescent; D000328:Adult; D001944:Breath Tests; D002648:Child; D002925:Ciliary Motility Disorders / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D059446:Heterotaxy Syndrome / Q000150:complications*; D006801:Humans; D008297:Male; D018715:Microscopy, Video; D008875:Middle Aged; D015999:Multivariate Analysis; D009569:Nitric Oxide / Q000378:metabolism; D010033:Otitis Media / Q000150:complications; D012130:Respiratory Hypersensitivity / Q000150:complications; D012140:Respiratory Tract Diseases / Q000150:complications; D055815:Young Adult
chemical_list: D009569:Nitric Oxide
keywords: ciliary dyskinesia; heterotaxy; nasal nitric oxide