Abstract

Abstract Background Compared with those born at term gestation, infants with complex congenital heart defects (CCHD) who were delivered before 37 weeks of gestational age and received neonatal open cardiac surgery (OHS) have poorer neurodevelopmental outcomes in early childhood. Specific details related to the neurodevelopmental outcome of these infants remain unpublished. Objectives To describe the growth, disability, functional, and neurodevelopmental outcome in early childhood of preterm infants (born at <37+0 weeks gestation) with CCHD and neonatal OHS. Design/Methods We studied all infants with CCHD who received OHS within 6 weeks of corrected age between 1996 and 2016. In the Western Canadian Complex Pediatric Therapies Follow-up Program, comprehensive neurodevelopmental assessments at a corrected age of 18-24 months were done by multidisciplinary teams at the original referral sites. In addition to demographic and clinical data, standardized age-appropriate outcome measures included physical growth with calculated Z-scores, disabilities including cerebral palsy, visual impairment, sensorineural hearing loss; adaptive function (Adaptive Behavioural Assessment System-II); and cognitive, language, and motor skills (Bayley Scales of Infant and Toddler Development-III). Results From 1996 to 2016, 115 preterm infants (34±2 weeks gestation, 2339±637g, 64% males) with CCHD had OHS with 11(10%) deaths before first discharge and 21 (18%) by 2 years. Prior to the first surgery, 7 (6%) neonates had cerebral injuries. Overall, 7 had necrotizing enterocolitis; none had retinopathy of prematurity. All 94 surviving infants received comprehensive evaluation at 2 years corrected age; Eighteen (19%) had congenital syndromes who had worse functional and neurodevelopmental outcomes compared to those (n=76) without syndromal abnormalities (SA) (Table). Conclusion For preterm neonates with CCHD and early OHS, the mortality was significant, but the short-term neonatal morbidity was not increased. Compared with published preterm outcomes, the early outcome suggests more cerebral palsy but not sensorineural hearing loss, and greater neurodevelopmental delay. This information is important for management care of the infants, parental counselling and the decision-making process.

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