Central nervous system outcomes in children with complex congenital heart disease

To provide a brief overview of our current understanding of the types of neurodevelopmental sequelae in congenital heart disease survivors and to review the most recent studies from the past year, which have focused on 4 interrelated issues: (1) outcome studies, (2) the mechanism and etiology of central nervous system injury in children with CHD, (3) perioperative monitoring for brain injury, and (4) strategies for neuroprotection during cardiac surgery.

As the number of survivors of surgery for complex congenital heart disease continues to rise, it is recognized that there is an increased incidence of adverse neurological outcomes in the survivors. In particular, a pattern similar to that seen in premature infants is emerging, including learning disabilities, behavioral abnormalities, inattention and hyperactivity. Imaging studies have revealed a high prevalence of structural brain abnormalities and periventricular leukomalacia, fetal and postnatal cerebral blood flow is abnormal, postnatal oxygen delivery is decreased, and intraoperative support techniques and postoperative low cardiac output are associated with cerebral hypoperfusion.

The causes of these late developmental abnormalities are most likely sequential, cumulative and multifactorial.