Advances in the management of infants with congenital heart disease ha
ve lead to a striking decrease in mortality. The most dramatic impact
has been in the newborn infant with complex and previously lethal hear
t disease. These heart lesions have become amenable to corrective proc
edures in the newborn period because of the development of support tec
hniques such as low-flow cardiopulmonary bypass and deep hypothermic c
irculatory arrest. However, these techniques have demonstrated their o
wn inherit risk for neurologic injury. Consequently, in recent years t
here has emerged a growing population of infants surviving congenital
heart disease only to manifest subsequent neurologic complications ori
ginating from injury in the hemodynamically unstable preoperative peri
od or periods of intraoperative hypoperfusion. The preservation of neu
rology function has emerged as the next frontier in the management of
congenital heart disease. Finally, neurologic dysfunction in the newbo
rn with cardiac disease may reflect associated brain malformations or
the combined cardiac and brain manifestations of inherited metabolic d
isease. The clinical features and mechanisms of brain injury are discu
ssed for these structural and metabolic cardiac diseases presenting in
the newborn infant.