Determinants of neuropsychological and behavioural outcomes in early childhood survivors of congenital heart disease

Aims

To evaluate the relative effect of cyanosis, surgical interventions and family processes on neuropsychological and behavioural outcomes in 4‐year‐old survivors of serious congenital heart disease (CHD).

Methods

90 children with a range of cyanotic and acyanotic conditions, who underwent either corrective or palliative surgery, completed a neuropsychological and behavioural evaluation. Families of participants were also profiled by evaluation of maternal mental health, worry, social support, parenting style and family functioning.

Results

Compromised neuropsychological outcomes were associated with a combination of cyanotic conditions and open‐heart surgery, but this was not exacerbated by having a complex, palliative, status. Both cyanotic and acyanotic conditions were associated with specific sensorimotor delays, regardless of method of the correction. Only children with complex conditions and palliative interventions seemed at risk of poor behavioural outcomes; indeed, children with cyanosis with complete repair showed favourable behavioural outcomes compared with controls. Multivariate analyses highlighted the sometimes greater relevance of family processes (eg parenting style, maternal mental health and worry), rather than disease or surgical factors, in predicting especially behavioural outcomes.

Conclusions

The findings (1) suggest a more complex relationship between cyanosis, surgical methods of correction, neuropsychological and behavioural outcomes than previously charted, (2) highlight that family processes may be aetiologically more important than disease and surgical factors, and (3) indicate specific targets for secondary prevention programmes for this at‐risk population.An emerging view suggests that children with cyanotic congenital heart disease (CHD) who have undergone surgical procedures involving circulatory arrest are at particular risk of both neuropsychological and behavioural problems.^1,2,3,4,5 However, some studies have found no such evidence or indeed, in terms of behavioural adjustment, suggested better outcomes than controls.^6,7,8 Moreover, where statistically significant differences have been found, clinical significance is sometimes dubious. Indices of intellectual functioning and behavioural adjustment in some studies have often been well within the normal range, with statistical significance sometimes determined by above‐average scores in control groups.^3,9,10Contributing to this ambiguity, studies have often used restricted samples of children with CHD (eg cases of transposition of great arteries) and have neglected to examine the mediating effects of maternal and family factors, which can covary with CHD.^6,8,11,12,13 This study includes measures of maternal adaptation and family functioning, in addition to cyanosis and surgical factors, in examining determinants of neuropsychological and behavioural outcomes in 4‐year‐old survivors of severe congenital heart defects. Children with cyanosis are divided into subgroups of those who had corrective versus palliative surgery only. These subgroups have often been merged in previous studies, but important differences are likely. Children without acyanosis are also subgrouped into those who underwent open or closed surgery. In this way, the importance of surgical procedures involving cardiopulmonary bypass, independent of cyanosis, may be considered. Neuropsychological and behavioural profiles across groups are first considered, followed by multivariate analyses of outcome determinants.