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 .
Clinical and Functional Developmental Outcomes in Neonates Undergoing Truncus Arteriosus Repair: A Cohort Study.
BACKGROUND Truncus arteriosus ( TA ) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired . The objective of this study was to assess the clinical and developmental outcomes in a prospect i ve cohort of patients who underwent TA repair .  METHODS All patients who underwent a TA repair between 1996 and 2012 were included . Follow-up clinical , neurologic , and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program data base . Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II , General Adaptive Composite ( GAC ) score . Survival and outcomes were compared between those with and without chromosomal abnormalities ( CA ) . Survival and freedom from reintervention were assessed by Kaplan-Meier analysis . RESULTS The study comprised 36 infants ( 19 male ) . CA was identified in 13 , with 22q11.2 deletion in 10 patients . Patients underwent TA repair at a median age of 10 days ; 5 patients underwent concomitant interrupted arch repair . There were 8 deaths , 2 of which occurred in the hospital . The 5-year survival was 79.4 % . Survival was similar between those with and without CA . At 5 years , freedom from reoperation was 77.2 % . The mean GAC was higher in the patients without CA ( 93.6 ± 12.8 vs 76.1 ± 13.1 , p = 0.0016 ) . CONCLUSIONS Patients with surgically repaired TA continue to have significant postoperative mortality . Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life . Functional developmental outcome in patients without CA is good , although it is significantly impaired in those with CA
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 .