Objective Magnetic Resonance Imaging (MRI) has defined neurologic abnormalities in infants with congenital heart disease (CHD) including pre-operative injury and delayed brain maturation. This study utilized qualitative scoring, cerebral biometry, and diffusion imaging to characterize pre-operative brain abnormalities in infants with CHD, including the identification of regions of greater vulnerability. Methods Sixty-seven infants with CHD had pre-operative MRI with analysis for brain injury by qualitative scoring and brain development by qualitative scoring, metrics and diffusion imaging. Results Qualitative abnormalities were common, with 42% of infants having pre-operative focal white matter lesions. Infants with CHD had smaller brain measures in the frontal lobe, parietal lobe, cerebellum and brainstem (p<.001); with the frontal lobe and brainstem displaying the greatest alterations (p<.001). Smaller brain size in the frontal and parietal lobes correlated with delayed white matter microstructure reflected by diffusion imaging. Conclusion Infants with CHD commonly display brain injury and delayed brain development. Regional alterations in brain size are present, with the frontal lobe and brainstem demonstrating the greatest alterations, which may reflect a combination of developmental vulnerability and regional differences in cerebral circulation.

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AIR is common among patients with heterotaxy syndrome and congenital heart disease. We recommend that patients with congenital heart disease and heterotaxy syndrome have routine elective abdominal imaging of their gastrointestinal tract at birth as part of their evaluation.