Abstract #2543

Validation of Semi-quantitative Brain Dysmaturation Score in Congenital Heart Disease: Correlated with Executive Function and Ciliary Motion

Vincent Kyu Lee^1,2, William Thomas Reynolds^2,3, Julia Wallace², Nancy Beluk², Subramanian Subramanian², Daryaneh Badaly⁴, Rafael Ceschin^2,3, Cecilia Lo⁵, and Ashok Panigrahy^1,2,3

¹Department of Bioengineering, University of Pittsburgh, Pittsburgh, PA, United States, ²Department of Radiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States, ³Department of Biomedical Informatics, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States, ⁴Learning and Development Center, Child Mind Institute, New York, NY, United States, ⁵Department of Developmental Biology, University of Pittsburgh, Pittsburgh, PA, United States

Synopsis

Keywords: Neuro, Adolescents, Congenital Heart Disease Neurodevelopment Brain Dysmaturation Evaluation Method

Motivation: Develop and validate point-of-care MRI-based evaluation method for scoring brain dysplasia/abnormality (BDS) in congenital heart disease (CHD) that incorporates morphological alterations and subcortical structures.

Goal(s): Further develop our BDS system, previously validated in infants with CHD, in the older pediatric and young adult CHD population.

Approach: Evaluate brain dysplasia from T1 and T2 structural MRI of CHD and control participants and compare differences. Correlate BDS with executive function outcomes and genetic ciliary motion (CM) abnormalities.

Results: CHD group had higher total and subcortical dysplasia, especially single ventricle CHD group. Higher BDS (greater dysplasia) correlated with poorer executive function outcomes and greater CM abnormality.

Impact: Our BDS method is sensitive to dysmaturational features in CHD and correlated with executive function outcomes and CM - genetic-basis of CHD pathogenesis. Since it employs common point-of-care MRI techniques, it could be adapted for wider application in CHD brain evaluation.

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