title: Brain Dysplasia Associated with Ciliary Dysfunction in Infants with Congenital Heart Disease.
abstract: OBJECTIVE
  To test for associations between abnormal respiratory ciliary motion (CM) and brain abnormalities in infants with congenital heart disease (CHD) STUDY DESIGN: We recruited 35 infants with CHD preoperatively and performed nasal tissue biopsy to assess respiratory CM by videomicroscopy. Cranial ultrasound scan and brain magnetic resonance imaging were obtained pre- and/or postoperatively and systematically reviewed for brain abnormalities. Segmentation was used to quantitate cerebrospinal fluid and regional brain volumes. Perinatal and perioperative clinical variables were collected.
  
  
  RESULTS
  A total of 10 (28.5%) patients with CHD had abnormal CM. Abnormal CM was not associated with brain injury but was correlated with increased extraaxial cerebrospinal fluid volume (P < .001), delayed brain maturation (P < .05), and a spectrum of subtle dysplasia including the hippocampus (P < .0078) and olfactory bulb (P < .034). Abnormal CM was associated with higher composite dysplasia score (P < .001), and both were correlated with elevated preoperative serum lactate (P < .001).
  
  
  CONCLUSIONS
  Abnormal respiratory CM in infants with CHD is associated with a spectrum of brain dysplasia. These findings suggest that ciliary defects may play a role in brain dysplasia in patients with CHD and have the potential to prognosticate neurodevelopmental risks.
authors: Department of Pediatric Radiology, Childrens Hospital of Pittsburgh of University of Pittsburgh Medical Center and University of Pittsburgh School of Medicine, Pittsburgh, PA; Department of Biomedical Informatics, University of Pittsburgh, Pittsburgh, PA. Electronic address: panigrahya@upmc.edu.
authors: Ashok
authors: A
authors: Panigrahy
authors: Department of Pediatric Radiology, Childrens Hospital of Pittsburgh of University of Pittsburgh Medical Center and University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Vincent
authors: V
authors: Lee
authors: Department of Pediatric Radiology, Childrens Hospital of Pittsburgh of University of Pittsburgh Medical Center and University of Pittsburgh School of Medicine, Pittsburgh, PA; Department of Biomedical Informatics, University of Pittsburgh, Pittsburgh, PA.
authors: Rafael
authors: R
authors: Ceschin
authors: Department of Pediatric Radiology, Childrens Hospital of Pittsburgh of University of Pittsburgh Medical Center and University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Giulio
authors: G
authors: Zuccoli
authors: Department of Pediatric Radiology, Childrens Hospital of Pittsburgh of University of Pittsburgh Medical Center and University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Nancy
authors: N
authors: Beluk
authors: Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Omar
authors: O
authors: Khalifa
authors: Department of Pediatrics, Division of Cardiology, Childrens Hospital of Los Angeles, Los Angeles, CA.
authors: Jodie K
authors: JK
authors: Votava-Smith
authors: Division of Pediatric Cardiology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Mark
authors: M
authors: DeBrunner
authors: Cardiac Intensive Care Division, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Ricardo
authors: R
authors: Munoz
authors: Cardiac Intensive Care Division, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Yuliya
authors: Y
authors: Domnina
authors: Division of Pediatric Cardiothoracic Surgery, Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Victor
authors: V
authors: Morell
authors: Division of Pediatric Cardiothoracic Surgery, Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Peter
authors: P
authors: Wearden
authors: Cardiac Intensive Care Division, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Joan
authors: J
authors: Sanchez De Toledo
authors: Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: William
authors: W
authors: Devine
authors: Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Maliha
authors: M
authors: Zahid
authors: Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors: Cecilia W
authors: CW
authors: Lo
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D001927:Brain Diseases / Q000150:complications / Q000453:epidemiology*; D002925:Ciliary Motility Disorders / Q000150:complications*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D011446:Prospective Studies
keywords: choroid plexus; extraaxial CSF; hippocampus; motile cilia; olfactory