Brain Dysplasia Associated with Ciliary Dysfunction in Infants with Congenital Heart Disease

Overview

Journal J Pediatr

Specialty Pediatrics

Date 2016 Aug 31

PMID 27574995

Citations 17

Authors

Ashok Panigrahy

Vincent Lee

Rafael Ceschin

Giulio Zuccoli

Nancy Beluk

Omar Khalifa

Jodie K Votava-Smith

Mark DeBrunner

Ricardo Munoz

Yuliya Domnina

Victor Morell

Peter Wearden

Joan Sanchez de Toledo

William Devine

Maliha Zahid

Cecilia W Lo

Affiliations

Soon will be listed here.

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.

Citing Articles

Validation of a Paralimbic-Related Subcortical Brain Dysmaturation MRI Score in Infants with Congenital Heart Disease.

Reynolds W, Votava-Smith J, Gabriel G, Lee V, Rajagopalan V, Wu Y J Clin Med. 2024; 13(19).

PMID: 39407833 PMC: 11476423. DOI: 10.3390/jcm13195772.

Olfactory Bulb Volume and Asymmetry as Predictors of Executive Dysfunction in Adolescents with Congenital Heart Disease.

Racki A, Shah A, Slabicki R, Wallace J, Lee V, Ceschin R medRxiv. 2024; .

PMID: 39399014 PMC: 11469464. DOI: 10.1101/2024.09.24.24314159.

The role of primary cilia in congenital heart defect-associated neurological impairments.

Saric N, Ishibashi N Front Genet. 2024; 15:1460228.

PMID: 39175754 PMC: 11338889. DOI: 10.3389/fgene.2024.1460228.

Postnatal Brain Trajectories and Maternal Intelligence Predict Childhood Outcomes in Complex CHD.

Lee V, Ceschin R, Reynolds W, Meyers B, Wallace J, Landsittel D J Clin Med. 2024; 13(10).

PMID: 38792464 PMC: 11121951. DOI: 10.3390/jcm13102922.

Quantitative Magnetic Resonance Cerebral Spinal Fluid Flow Properties and Executive Function Cognitive Outcomes in Congenital Heart Disease.

Lee V, Reynolds W, Wallace J, Beluk N, Badaly D, Lo C medRxiv. 2024; .

PMID: 38699300 PMC: 11065010. DOI: 10.1101/2024.04.19.24306104.

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