Airway Ciliary Dysfunction and Sinopulmonary Symptoms in Patients with Congenital Heart Disease

• DOI: 10.1513/annalsats.201405-222oc
• Publication Date: 2014-10-10T20:29:26Z
• Authors: Andrea S. Garrod; Maliha Zahid; Xin Tian; Richard J. Francis; Omar Khalifa; William Devine; George C. Gabriel; Linda Leatherbury; Cecilia W. Lo
• Keywords: Heterotaxy; Kartagener Syndrome

Rationale: Patients with congenital heart disease heterotaxy exhibit a high prevalence of abnormal airway ciliary motion and low nasal nitric oxide, characteristics associated primary dyskinesia, reflection the role motile cilia in clearance left–right patterning.Objectives: To assess potential broader clinical significance dysfunction disease, we assessed versus respiratory symptoms patients or without heterotaxy.Methods: broad spectrum were recruited (n = 218), 39 heterotaxy. Nasal oxide measurements biopsies for video microscopy conducted. Sinopulmonary reviewed by questionnaire.Measurements Main Results: A defects (51.8%) borderline levels (35.5%) observed showed increased sinopulmonary symptoms, most seen those both oxide. Multivariate analysis that more important determining risk than status.Conclusions: This was symptoms. These findings suggest may benefit from screening implementation medical interventions to reduce morbidities.