542 - Variables affecting neurodevelopmental outcome in infants with critical congenital heart disease

Publication Number: 542.7013

Elizabeth C. Loughman, Children's Mercy Hospitals and Clinics, Kansas City, MO, United States; Julie Weiner, Children's Mercy Hospitals and Clinics, Kansas City, MO, United States; Marcie Files, Children's Mercy Kansas City, kansas city, MO, United States

Background: The mortality of cardiac conditions in infancy is improving with advances in surgical planning and medical management. Neurologic morbidity remains significant. Clinically correlating an infant with their neurologic exam, MRI/EEG, and subsequent developmental testing remains a challenge. Counseling regarding neurologic outcomes after cardiac intervention is difficult due to the wide range of findings that can sometimes be clinically irrelevant.

Objective: : The goal of this study is to identify factors associated with poor neurodevelopmental outcomes in infants with critical congenital heart defects (CCHD) as assessed by the Bayley Scales of Infant and Toddler Development Edition IV (Bayley).

Design/Methods: This is a retrospective single site study performed at a freestanding children’s hospital with delivery services. Neonates were inborn or admitted to the neonatal/cardiac intensive care units following diagnosis of CCHD over a 5-year period who required cardiac surgical procedures during their initial hospital stay. Patients were sampled from databases in the heart center and the Children’s Hospital Neonatal Consortium. Neonates were stratified by single vs biventricular repair and Norman category of heart defect (A, B, or C). Primary outcomes included neurologic exam, MRI, and EEG at discharge and 12- and 24-month Bayleys.

Results: 383 neonates were included in analysis. 57.2% were male with an average gestational age of 39 weeks and average birthweight of 3.2kg. The average length of stay was 36 days. Of the entire sample, 36.3% had Bayleys performed at 12 months and 25.8% at 24 months. 38% of the entire sample underwent a single ventricle repair. 39% were classified as Norman A, 41% Norman B, and 19% Norman C. 40% of Bayleys were abnormal, with highest impairment in gross motor skills and expressive language. Of the 145 infants with EEGs, 17% showed seizures or focal slowing. Of these abnormal EEGs, approximately 45% had an abnormal Bayley. Of the 146 infants with MRIs, 58% showed ischemia, infarct, atrophy, stroke, or hemorrhage. The odds in favor of an abnormal Bayley at 12 months was 3.11 times higher among infants with abnormal MRIs (95% CI 1.22-7.94, d= 0.63). 6.5% had an abnormal neurologic exam, most commonly hypotonia. Of these abnormal neurologic exams, 73% had abnormal 12-month Bayleys and 87.5% had abnormal 24-month Bayleys.

Conclusion(s): Abnormal neurologic exams, MRI, and EEG at discharge can be associated with abnormal Bayleys in childhood. This helps in counseling families and providing long term neurodevelopmental follow up.

Abnormal Bayley exams and associated patient characteristics
Odds ratio via univariable logistic regression of several infant factors showing higher or lower odds of abnormal Bayley, stratified by 12 and 24 month testing.

Abnormal 12 month Bayleys and associated patient characteristics
Factors appearing in bold were statistically significant to represent a higher likelihood of abnormal Bayley testing at 12 months.

Abnormal 24 month Bayleys and associated patient characteristics
Factors appearing in bold were statistically significant to represent a higher likelihood of abnormal Bayley testing at 24 months.

Abnormal Bayley exams and associated patient characteristics
Odds ratio via univariable logistic regression of several infant factors showing higher or lower odds of abnormal Bayley, stratified by 12 and 24 month testing.

Abnormal 12 month Bayleys and associated patient characteristics
Factors appearing in bold were statistically significant to represent a higher likelihood of abnormal Bayley testing at 12 months.

Abnormal 24 month Bayleys and associated patient characteristics
Factors appearing in bold were statistically significant to represent a higher likelihood of abnormal Bayley testing at 24 months.