Congenital cardiac anomalies and white matter injury

• Complex congenital heart disease (CHD) is often associated with developmental delay and behavioral problems.
• Currently, the neurological deficits displayed by CHD patients are irreversible.
• Animal studies offer powerful avenues to understand CHD brain impairments.

Cardiac abnormalities are the most common birth defects. Derangement of circulatory flow affects many vital organs; without proper supply of oxygenated blood, the brain is particularly vulnerable. Although surgical interventions have greatly reduced mortality rates, patients often suffer an array of neurological deficits throughout life. Neuroimaging provides a macroscopic assessment of brain injury and has shown that white matter (WM) is at risk. Oligodendrocytes and myelinated axons have been identified as major targets of WM injury, but still little is known about how congenital heart anomalies affect the brain at the cellular level. Further integration of animal model studies and clinical research will define novel therapeutic targets and new standards of care to prevent developmental delay associated with cardiac abnormalities.