Cardiovascular Journal of Africa: Vol 33 No 3 (MAY/JUNE 2022)

CARDIOVASCULAR JOURNAL OF AFRICA • Volume 33, No 3, May/June 2022 150 AFRICA Although assessment at an earlier time point following diagnosis would have been preferred, in our experience, this would not have been feasible at the study site. Children were seldom referred to neurodevelopmental services pre-operatively, and the increased hospital costs and inconvenience to families associated with earlier pre-operative hospital admission was not found to be acceptable. The majority of parents (60%) in this study experienced clinically significant parenting stress on the admission of their child for cardiac surgery, making it more challenging to engage with them in the pre-operative neurodevelopmental assessment.49,54,55 This was often parents’ first exposure to a developmental component of their child’s cardiac care, which may have contributed to their stress at the time. It is possible that the children could have taken on their parents’ stress and anxiety, which may also have negatively affected their developmental performance during testing. Assessment on the Bayley-III required the children to be alert and able to tolerate physical handling and position changing.53,56 Children included in the current study were medically stable, but most were malnourished. Current malnutrition practice guidelines however deem it safe to perform neurodevelopmental assessment when the child is medically stable.57,58 Child-related barriers that prevented pre-operative neurodevelopmental assessment in this study included critical illness and the need for emergency cardiac surgery. This is consistent with barriers to pre-operative neurodevelopmental assessment identified in previous studies.21-23 Pre-operative developmental performance of children with CHD without DS was considered to be average and the prevalence of pre-operative developmental delays was within the estimated range of 13 to 16% for the general population.59 Motor delays were found to be most prevalent pre-operatively, which is consistent with the published evidence on early developmental outcomes in children with CHD.20,56 The cause of the motor delays are likely multifactorial, including hypotonia, periods of pre-operative immobilisation and maternal overprotection.12,20,56,60 In addition, cardiovascular compromise and related limited physical endurance may also have compromised the acquisition of motor skills.12,56,61 Pre-operative developmental performance for childrenwithDS was found to be at risk, or in most cases, delayed. Developmental performance was significantly poorer for children with DS across all developmental domains. This was to be anticipated as DS is known to be the leading genetic cause of intellectual and developmental disability in children.38,62 Motor delays were also most prevalent in children with DS, attributable to the marked hypotonia and poor postural and antigravity control associated with DS.37,38 This was likely superimposed on other known causes of motor delay in children with CHD, explaining the greater extent of the motor delays.12,63,64 Growth failure was significantly associated (p = 0.04) with pre-operative motor delay. Malnutrition is associated with muscle weakness and poor physical endurance, which would prevent children from engaging in typical age-appropriate developmental activities.12 Several studies have confirmed the positive association between growth failure and delayed gross motor development in children with CHD.9,65,66 Pre-operative neurodevelopmental assessment proved valuable in identifying children, both with and without DS, with developmental delays. This allowed for the prioritisation of postoperative neurodevelopmental follow up and early referral for individualised rehabilitation therapies to address developmental delays, as well as the provision of appropriate education and advice to parents. Referral to early rehabilitation therapies during hospitalisation or at hospital discharge can be discussed with parents in the context of their child’s neurodevelopmental assessment findings, which may improve the uptake of these important services. The proportion of children without and with DS at risk for and presenting with developmental delays, qualifying them for referral to rehabilitation therapies, was comparable at six months post cardiac surgery. Importantly, most of the children identified as being at risk or delayed pre-operatively were also identified to be so at six months post cardiac surgery, which would suggest pre-operative neurodevelopmental assessment findings may potentially be predictive of shorter-term post-operative outcomes. The changing developmental profile of children with CHD without and with DS over time and with increasing age in the current study affirms the need for regular ongoing developmental assessment throughout childhood.20 Strengths and limitations This is the first study to determine the pre-operative neurodevelopmental statusof youngchildrenwithCHDundergoing cardiac surgery in South Africa, and to report on the feasibility, clinical value, and format of pre-operative neurodevelopmental assessment from a developing-country perspective. The findings of this study however need to be considered in light of several limitations. The outcomes reported are for a single cardiac centre, making the findings specific to this population, and not necessarily generalisable to the CHD population at large. The study was limited by a small sample size, and therefore small sub-group size, which does not allow for definitive conclusions to be drawn from the findings. Child-related and particularly environmental barriers challenged recruitment and the completion of pre-operative neurodevelopmental assessments. Recommendations Despite the challenges of performing pre-operative neurodevelopmental assessment in medically stable children with CHD, the potential benefits cannot be overlooked in the South African context where children often wait for extended periods before undergoing cardiac surgery. It is recommended that medically stable children with CHDbe referred for developmental screening after diagnosis and while awaiting cardiac surgery. It would be optimal to perform this screening, where possible, at a routine clinic visit prior to hospital admission for cardiac surgery. It is advised that for clinical practice, pre-operative developmental screening makes use of a brief standardised screening test or a parental questionnaire such as the Ages & Stages Questionnaires (third edition) (ASQ-3).2,67 Clinicians should clearly explain the value of pre-operative developmental assessment to parents, and adequate reassurances and support should be provided to reduce their anxiety. Children identified as being at risk for or presenting with developmental delays should be referred for the initiation of individualised rehabilitation therapies adapted to the child’s clinical condition while awaiting surgery. Therapy should be

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