Long term behavioural outcome after neonatal arterial switch operation for transposition of the great arteries

Abstract

Aims: To evaluate behavioural outcome and quality of life in children aged 8-14 years after neonatal arterial switch operation for transposition of the great arteries. Methods: Sixty children operated as neonates with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass were evaluated at age 7.9-14.3 years by the Child Behaviour Checklist (CBCL) and the Inventory for the Assessment of the Quality of Life in Children and Adolescents (IQCL). Results: Parent reported behavioural outcome on all CBCL problem and competence scores was worse, whereas quality of life on self reported IQCL scores was not reduced compared to the normal population. On multivariate analysis, severe preoperative hypoxia was related to parent reported social problems; peri-and postoperative cardiocirculatory insufficiency was associated with internalising, externalising, attention, and total behavioural problems. Reduced expressive language was associated with total behavioural problems, and poor academic achievement was related to parent reported deficits in school performance. Impaired neurological status and reduced endurance capacity both predicted self reported stress by illness. Conclusions: The neonatal arterial switch operation with combined circulatory arrest and low flow bypass is associated with parent reported long term behavioural impairment, but not with self reported general reduction in quality of life. This discrepancy may be a result of different perception of illness. In our experience, increased risk of long term psychosocial maladjustment after neonatal corrective cardiac surgery is related to the presence of neurological impairment and reduced endurance capacity.

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

“…164 Behavioural and socialisation problems, as reported by parents, are correlated with poorer school performance and self-reported quality of life in young people who have had repair of TGA in infancy. 190 A child's body image may alter with chronic illness 192 and children's understanding of their own illness is often poor. 193 School-age children with congenital heart defects have been found to need more support from teachers in coping with their school absences, peer relationships and participation in educational activities.…”

“…187 Behaviour problems are more likely to arise in children with heart malformations than in the normal population and may be more common in cyanotic heart defects, in children with greatest physical limitation and after cardiac arrest. 105,169,182,[188][189][190][191] Socialisation skills were poor in >50% of preschool children who had had surgery for congenital heart defects. 164 Behavioural and socialisation problems, as reported by parents, are correlated with poorer school performance and self-reported quality of life in young people who have had repair of TGA in infancy.…”

Preventing Brain Injury in Newborns With Congenital Heart Disease

“…21 Recently, it has been recognized that more than half of newborns with CHD have clinical evidence of neurological abnormalities on examination before surgery and that these abnormalities are a significant risk factor for later neurodevelopmental impairment. 18,36,38 Role of Brain Imaging in Identifying Mechanism of Brain Injury MRI studies of newborns with CHD have shown that up to 40% have preoperative brain injuries. 35,39 Postoperative MRI showed that an additional third of those studied acquired new injuries such that, overall, more than half of those studied had acquired brain lesions.…”

“…16 In other cohorts, children with TGA were more likely to have abnormal neurological examination findings, learning disabilities, and behavioral disorders compared with population norms. 9,[17][18][19] Adverse neurodevelopmental outcomes are not restricted to newborns with TGA; motor and global developmental delays have been reported in children with multiple types of CHD. 14 For example, the incidence of major disabilities in survivors with hypoplastic left heart syndrome exceeds 60%.…”

Effect of balloon atrial septostomy on cerebral oxygenation in neonates with transposition of the great arteries

“…Although mortality is low, neonates with TGA are at risk of impaired neurodevelopmental outcome. Long-term follow-up demonstrates that 30-50% of school-aged children with TGA show some form of developmental delay, including fine and gross motor delay, impaired visuomotor integration, language and speech difficulties, and behavioral problems (2)(3)(4)(5).…”