Introduction: Neurodevelopmental disability in neonate with congenital heart defects has largely been attributed to complications of open heart surgery, without consideration of preoperative neurologic status. So the controversy exists regarding the integrity of the nervous system in the newborn with a congenital heart defect (CHD) who must undergo corrective or palliative open heart surgery.

Aim: To determine the neurobehavioral status of newborns with CHD.

Material and Methods: In this prospective study, a new neonatal neurobehavioral examination (NNE) was conducted independently in a consecutive series of 40 neonates with congenital heart defects. The NNE consists of 27 items divided into three sections: 1) tone and motor patterns, 2) primitive reflexes, and 3) behavioral responses. Neurologic examinations were carried out as well. Cardiorespiratory status was determined at the time of assessment.

Results: Neurobehavioral abnormalities were documented in 62% of newborns and included hypotonia, hypertonia, jitteriness, motor asymmetry, and absent suck. Poor state regulation (n=16) and decreased feeding efficiency (13) were commonly observed as well. In addition, 10% neonates had seizures and 24% were microcephalic. The overall likelihood of neurobehavioral abnormalities was not enhanced by indicators of cardiorespiratory compromise. Interestingly, newborns with acyanotic congenital heart defects were more likely to demonstrate neurologic compromise than were those with cyanotic defects.

Conclusion: Neuromotor abnormalities are common in neonates with congenital heart defects. These developmental deficits are under-recognized clinically, and may suggest the need for early systematic developmental screening in neonates with CHD.