Abstract 16570: Respiratory Ciliary Motion Defect Predict Regional Brain Abnormalities and Increased Extra Axial CSF Fluid in Neonates With Complex Congenital Heart Disease
Objectives: We hypothesized congenital heart disease (CHD) patients with respiratory ciliary motion (CM) defects may have increased prevalence of brain abnormalities.
Background: We recently showed CHD patients have a high prevalence of airway motile cilia dysfunction. In the brain, motile cilia in the ependyma mediate cerebrospinal fluid (CSF) flow required for neuronal cell migration/neurogenesis. Cilia are also found in the choroid plexus and are required for maintenance of neural progenitors. CHD patients with airway motile ciliary dysfunction may be at increased risk of neurocognitive abnormalities.
Methods and Results: 40 neonates with complex CHD underwent preoperative cranial US, volumetric MRI and respiratory ciliary motion (CM) assessment with high-speed videomicroscopy of nasal biopsies. CM was scored on a scale of 1 to 4, with 1 indicating normal CM, ranging to 4 for highly abnormal CM. Multivariate logistical regression of brain measurements with CM scores revealed significant correlation between respiratory CM defects and brain abnormalities (right panel, p<0.05), including hippocampal dysplasia/hypoplasia, increased extra-axial fluid, choroid plexus abnormalities (hypertrophy/nodularity) and corpus callosum dysplasia. We observed marginal correlations of CM defects with micrognathia, olfactory abnormalities and brainstem dysplasia. Correlation between volumetric increase in CSF and CM defects was observed using a neonatal brain segmentation template (r=0.64, p<0.007) (left panel).
Conclusions: Airway ciliary dysfunction is associated with structural brain abnormalities in neonates with complex CHD including: (1) subtle brain dysplasia in the olfactory bulb/hippocampus; (2) choroid plexus abnormalities and (3) increased extra-axial CSF fluid. These clinical translational studies suggest a potential unifying role for the cilia in contributing to both structural heart disease and brain abnormalities in CHD patients.
Author Disclosures: A. Panigrahy: None. R. Ceschin: None. V. Lee: None. N. Beluk: None. O. Khalifa: None. G. Zuccoli: None. R. Munoz: None. Y. Domnina: None. P. Wearden: None. V. Morell: None. M. Zahid: None. C.W. Lo: None.
- © 2014 by American Heart Association, Inc.