Abstract

Neurodevelopmental disability in children with congenital heart defects has largely been attributed to complications of open heart surgery, without consideration of preoperative neurologic status. The objective of this ongoing prospective study is to compare baseline and acute postoperative neuromotor performance in newborns and infants with congenital heart defects. The Einstein Neonatal Neurobehavioral Assessment Scale was performed on all newborns, and infants were evaluated using the Peabody Developmental Motor Scale. Neurologic examinations were carried out as well. Cardiorespiratory status was determined at the time of assessment. To date, 48 newborns and 44 infants have been recruited. Mean age at baseline evaluation was 3.4 days (+/- 5.0) for newborns and 6.8 months (+/- 6.0) for infants. Neurobehavioral abnormalities were documented in 57% of newborns prior to surgery and included hypotonia, hypertonia, jitteriness, motor asymmetry, and absent suck. Poor state regulation (n=28) and decreased feeding efficiency (15) were commonly observed as well. The neurologist recorded comparable abnormalities in 52%. In addition, 2 neonates had seizures, 35% were microcephalic and 15% were macrocephalic. Neonates with acyanotic heart defects were more likely (p=.005) to demonstrate neurologic compromise. There was a significant association between baseline and postoperative assessment (Fisher Exact p=.0329), with only 17% deteriorating neurologically following surgery. Baseline neuromotor abnormalities were observed in 42% of infants and included hypotonia, motor delay, asymmetry, choreoathetosis, poor behavioral responses and feeding difficulties. Microcephaly was noted in 31%. Postoperatively, neuromotor status remained largely unchanged (Fisher Exact p=.0028), with only 3 children manifesting new neurologic abnormalities. Indicators of cardiorespiratory compromise (oxygen saturation, tachypnea, ventilation, congestive heart failure) at baseline assessment did not correlate with neuromotor performance. Neuromotor abnormalities are common in infants with congenital heart defects, and are evident prior to surgery in the majority of cases. These developmental deficits are under-recognized clinically, and may suggest the need for routine developmental screening in this population.

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