Structural evidence of injury or malformation in the brains of children with congenital heart disease.

Neurological and developmental deficits are common in children with congenital heart disease (CHD). These are due to multiple factors that include the etiology of the CHD, the effects of abnormal cardiovascular function, and the possible sequelae of open heart surgery. CHD is frequently part of a multiple malformation syndrome that includes the brain. The causes of these syndromes include known or putative genetic defects. Abnormal cardiovascular function may be associated with poor brain growth, embolic infarction, cerebrovascular thrombosis, and abscess formation. Perioperative neurological complications include diffuse hypoxic-ischemic injury (particularly in neonates who undergo more than 45 to 60 minutes of hypothermic circulatory arrest), cerebral macro- and micro-emboli, dural sinus thrombosis, and cerebral hemorrhage. Neuroimaging, especially magnetic resonance imaging, is a useful prognostic instrument, can easily display gross congenital and acquired lesions, and should be performed preoperatively in addition to genetic studies. In some instances poor brain function may not be predicted unless slow head growth or microcephaly is present and thorough preoperative neurodevelopmental evaluation is encouraged.