Perioperative risk factors were inconsistently associated with developmental outcomes .
INTERPRETATION The literature on children undergoing surgery in early infancy suggests that infants with a single ventricle are at highest risk of adverse developmental outcomes .
WHAT THIS PAPER ADDS Children with complex congenital heart disease ( CHD ) are at increased risk of impaired developmental outcome .
Children with single-ventricle CHD have worse outcomes than children with two-ventricle CHD .
Children with two-ventricle CHD gradually grow out of their initial developmental impairment .
Perioperative factors are inconsistently associated with outcome
AIM ( 1 ) To systematic ally review the literature on developmental outcomes from infancy to adolescence of children with complex congenital heart disease ( CHD ) who underwent early surgery ; ( 2 ) to run a meta-regression analysis on the Bayley Scales of Infant Development , Second Edition Mental Developmental Index and Psychomotor Developmental Index ( PDI ) of infants up to 24 months and IQs of preschool-aged children to adolescents ; ( 3 ) to assess associations between perioperative risk factors and outcomes .
Brain maturation is delayed in infants with complex congenital heart defects.
OBJECTIVE Small head circumferences and white matter injury in the form of periventricular leukomalacia have been observed in population s of infants with severe forms of congenital heart defects . This study tests the hypothesis that congenital heart defects delay in utero structural brain development . METHODS Full-term infants with hypoplastic left heart syndrome or transposition of the great arteries were prospect ively evaluated with preoperative brain magnetic resonance imaging .  Patients with independent risk factors for abnormal brain development ( shock , end-organ injury , or intrauterine growth retardation ) were excluded . Outcome measures included head circumferences and the total maturation score  on magnetic resonance imaging .  Total maturation score is a previously vali date d semiquantitative anatomic scoring system used to assess whole brain maturity . The total maturation score evaluates 4 parameters of maturity : ( 1 ) myelination , ( 2 ) cortical infolding , ( 3 ) involution of glial cell migration b and s , and ( 4 ) presence of germinal matrix tissue . RESULTS The study cohort included 29 neonates with hypoplastic left heart syndrome and 13 neonates with transposition of the great arteries at a mean gestational age of 38.9 + /- 1.1 weeks .  Mean head circumference was 1 st and ard deviation below normal . The mean total maturation score for the cohort was 10.15 + /- 0.94 , significantly lower than reported normative data in infants without congenital heart defects , corresponding to a delay of 1 month in structural brain development . CONCLUSION Before surgery , term infants with hypoplastic left heart syndrome and transposition of the great arteries have brains that are smaller and structurally less mature than expected . This delay in brain development may foster susceptibility to periventricular leukomalacia in the preoperative , intraoperative , and postoperative periods
Perioperative risk factors were inconsistently associated with developmental outcomes .
INTERPRETATION The literature on children undergoing surgery in early infancy suggests that infants with a single ventricle are at highest risk of adverse developmental outcomes .
WHAT THIS PAPER ADDS Children with complex congenital heart disease ( CHD ) are at increased risk of impaired developmental outcome .
Children with single-ventricle CHD have worse outcomes than children with two-ventricle CHD .
Children with two-ventricle CHD gradually grow out of their initial developmental impairment .
Perioperative factors are inconsistently associated with outcome
AIM ( 1 ) To systematic ally review the literature on developmental outcomes from infancy to adolescence of children with complex congenital heart disease ( CHD ) who underwent early surgery ; ( 2 ) to run a meta-regression analysis on the Bayley Scales of Infant Development , Second Edition Mental Developmental Index and Psychomotor Developmental Index ( PDI ) of infants up to 24 months and IQs of preschool-aged children to adolescents ; ( 3 ) to assess associations between perioperative risk factors and outcomes .