Abstract

Background: Compared with those born at term gestation, infants with complex congenital heart defects (CCHD) who were delivered before 37 weeks gestational age and received neonatal open-heart surgery (OHS) have poorer neurodevelopmental outcomes in early childhood. We aimed to describe the growth, disability, functional, and neurodevelopmental outcomes in early childhood of preterm infants with CCHD after neonatal OHS. Prediction models were evaluated at various timepoints during hospitalization which could be useful in the management of these infants.Study Design: We studied all preterm infants with CCHD who received OHS within 6 weeks of corrected age between 1996 and 2016. The Western Canadian Complex Pediatric Therapies Follow-up Program completed multidisciplinary comprehensive neurodevelopmental assessments at 2-year corrected age at the referral-site follow-up clinics. We collected demographic and acute-care clinical data, standardized age-appropriate outcome measures including physical growth with calculated z-scores; disabilities including cerebral palsy, visual impairment, permanent hearing loss; adaptive function (Adaptive Behavior Assessment System-II); and cognitive, language, and motor skills (Bayley Scales of Infant and Toddler Development-III). Multiple variable logistic or linear regressions determined predictors displayed as Odds Ratio (OR) or Effect Size (ES) with 95% confidence intervals.Results: Of 115 preterm infants (34 ± 2 weeks gestation, 2,339 ± 637 g, 64% males) with CCHD and OHS, there were 11(10%) deaths before first discharge and 21(18%) deaths by 2-years. Seven (6%) neonates had cerebral injuries, 7 had necrotizing enterocolitis; none had retinopathy of prematurity. Among 94 survivors, 9% had cerebral palsy and 6% had permanent hearing loss, with worse outcomes in those with syndromic diagnoses. Significant predictors of mortality included birth weight z-score [OR 0.28(0.11,0.72), P = 0.008], single-ventricle anatomy [OR 5.92(1.31,26.80), P = 0.021], post-operative ventilation days [OR 1.06(1.02,1.09), P = 0.007], and cardiopulmonary resuscitation [OR 11.58 (1.97,68.24), P = 0.007]; for adverse functional outcome in those without syndromic diagnoses, birth weight 2,000–2,499 g [ES −11.60(−18.67, −4.53), P = 0.002], post-conceptual age [ES −0.11(−0.22,0.00), P = 0.044], post-operative lowest pH [ES 6.75(1.25,12.25), P = 0.017], and sepsis [ES −9.70(−17.74, −1.66), P = 0.050].Conclusions: Our findings suggest preterm neonates with CCHD and early OHS had significant mortality and morbidity at 2-years and were at risk for cerebral palsy and adverse neurodevelopment. This information may be important for management, parental counseling and the decision-making process.

Highlights

  • METHODSInfants with complex congenital heart defects (CCHD) who have open-heart surgery (OHS) during the neonatal period are at risk for mortality and neurodevelopmental morbidity [1, 2]

  • From September 1996 to December 2016, all 115 preterm infants with CCHD who received OHS at ≤6 weeks corrected age at the Stollery Children’s Hospital were registered. These 115 children made up 10.7% of all children at age 6 weeks and under having complex cardiac surgery with cardiopulmonary bypass (CPB) at this institution during this time

  • This study adds “gestation-related information” to the mortality and developmental outcome information of preterm neonates with CCHD [5,6,7,8,9,10,11,12, 24, 25], and may be useful for the in-hospital management of patients and families including but not limited to (a) the timing of surgical correction, (b) the identification and correction of risk factors that are associated with adverse neurodevelopmental outcomes, (c) parental counseling on the course, care plan, possible outcomes, and importance of follow-up and early interventions, and (d) informing decision-making so clinicians have a better understanding of the course and outcome of these critically-ill preterm neonates

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Summary

Introduction

METHODSInfants with complex congenital heart defects (CCHD) who have open-heart surgery (OHS) during the neonatal period are at risk for mortality and neurodevelopmental morbidity [1, 2]. Despite the adverse outcome compared with that of term infants, there is little information available regarding “gestationrelated details” and predictors of outcomes in early childhood of preterm neonates with CCHD and early OHS at “different time points during hospitalization.” This information is important for management of the infants, parental counseling and informing the decision-making process. The secondary aims of the study included the identification of risk factors for mortality and adverse functional outcomes at each of five time points that we believe are important moments of clinical decision-making and family counseling: (i) before and at birth, (ii) pre-operative, (iii) day 1 post-operatively, (iv) post-operatively day 5, and (v) at first hospital discharge Compared with those born at term gestation, infants with complex congenital heart defects (CCHD) who were delivered before 37 weeks gestational age and received neonatal open-heart surgery (OHS) have poorer neurodevelopmental outcomes in early childhood. Prediction models were evaluated at various timepoints during hospitalization which could be useful in the management of these infants

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