1

[Original article: 4 tables; 1 online table]

Neurodevelopmental and health-related quality-of-life outcomes in adolescence

after surgery for congenital heart disease in infancy

JANE M WOTHERSPOON1

KAREN J EAGLESON2

LINDA GILMORE3

BENJAMIN AULD2

ANNE HIRST1

SUSAN JOHNSON2

CHRISTIAN STOCKER2,4

HELEN HEUSSLER4,5

ROBERT N JUSTO2,4

1 School of Psychology and Counselling, Queensland University of Technology,

Brisbane; 2 Queensland Paediatric Cardiac Service, Children’s Health Queensland,

Brisbane; 3 Faculty of Education, School of Cultural and Professional Learning,

Queensland University of Technology, Brisbane; 4 Faculty of Medicine, University of

Queensland, Brisbane; 5 Child Development Program, Children’s Health Queensland,

Brisbane, Australia.

Correspondence to Jane Wotherspoon, School of Psychology and Counselling,

Queensland University of Technology, Victoria Park Road, Kelvin Grove, 4059,

Brisbane, Australia. E-mail: jane.wotherspoon@connect.qut.edu.au

PUBLICATION DATA

Accepted for publication 00th Month 2019.

Published online 00th Month 2019.

ABBREVIATIONS

CHD Congenital heart disease

HRQoL Health-related quality of life

[Abstract]

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AIM To assess outcomes in adolescence after surgery for congenital heart disease in

infancy. Domains analysed included cognition and executive function, social and

emotional well-being, adaptive behaviour, academic achievement, and health-related

quality of life.

METHOD Twenty-one participants (10 males, 11 females) ranged in age from 14 to

17 years (mean 15y 4.8mo, SD 8.4mo). Twenty had biventricular repairs. All were

classified as New York Heart Association class I. Measures included: Wechsler

Intelligence and Achievement scales; Wide Range Assessment of Memory and

Learning, Second Edition; California Verbal Learning Test – Children’s Version;

Behaviour Rating Inventory of Executive Function; Conners, Third Edition; Adaptive

Behavior Assessment System, Second Edition; Behavior Assessment System for

Children, Second Edition; Rey–Osterrieth Complex Figure; and Pediatric Quality of

Life Inventory.

RESULTS Outcomes were significantly lower (p≤0.01) than population norms for

processing speed, mathematical achievement, attention, and visual–spatial ability.

Participants reported more frequent learning problems but more positive family

relations. Health-related quality of life was significantly lower across most domains

by self- and parent-proxy report.

INTERPRETATION Individuals with congenital heart disease may experience

difficulties across a range of domains. These findings emphasize the importance of

comprehensive screening, early intervention, and long-term follow-up, as deficits may

extend into young adulthood.

[First page footer]

© 2019 Mac Keith Press

DOI: 10.1111/dmcn.xxxxx

[Left page footer]

Developmental Medicine & Child Neurology 2019, 61: 000–000

[Right page footer]

Neurodevelopmental Outcomes after CHD Surgery Jane Wotherspoon et al.

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What this paper adds

Identified cognitive, learning, and attentional impairments in adolescents after

congenital heart disease surgery in infancy.

Combined self-report, caregiver report, and laboratory tasks in a

comprehensive neurodevelopmental assessment protocol.

Health-related quality of life was lower across most domains.

[Main text]

A significant majority (85–95%) of children born with congenital heart disease

(CHD) now survive to adulthood,1–3 with research attention consequently turning to

long-term neurodevelopmental outcomes.4 This population is at risk for

neurodevelopmental deficits in social and emotional functioning,5 executive

function,6–8 visual perception,9 motor skills,10 cognition and academic outcomes,4,11,12

and poorer health-related quality of life (HRQoL).13 Some researchers have proposed

that deficits can emerge as children age, with a recent study finding difficulties

increased significantly across various domains as individuals with CHD reached

upper primary school.14

Research suggests impaired executive function, encompassing processes such

as attention, inhibition, cognitive flexibility, and metacognition, is associated with

CHD.1,6,14,15 While intelligence is often within the ‘normal’ range, individuals with

CHD can struggle to coordinate cognitive skills in pursuit of complex goals.16 The

ability to work towards higher-order goals is foundational to achieving key

developmental outcomes in adolescence, including academic and employment success

and positive peer and family relationships.

Mechanisms for the emergence of neurodevelopmental impairment beyond

infancy in individuals with CHD are still being explored. Some researchers have

suggested lower-level skills are less affected in this population but that difficulties

become apparent when integration of such skills is required.14,15 Early insult to the brain

may impair networks that are less likely to affect lower-level skills and functions that

emerge in infancy but that are essential for the integration of neural functions that

occurs in later development.17 Alternative explanations for later emergence of deficits

include the suggestion that assessment tools for older children and adolescents can

better differentiate between those with CHD and the general population.17 Family risk

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factors, such as parent psychological well-being, perceptions of impairment, and

attachment, may also directly influence neurodevelopment, or interact with

neurological injuries over time to increase impairment.18–20

While adverse neurodevelopmental outcomes for young children with CHD

have been reported,13,21,22 fewer studies have evaluated later impact using a

comprehensive battery of tests, including both clinical measures and self- and proxy-

report measures of executive function.1,4,5,8 It has been suggested that relying on only

one measure of executive function can fail to capture everyday functioning,1 and result

in contradictory findings.5

This study aimed to assess executive function, cognition, academic

performance, emotional and behavioural well-being, and HRQoL in adolescents who

underwent cardiopulmonary bypass surgery before 6 months of age between 1999 and

2001. Initial enrolment criteria included no identified neurological abnormality or

syndromic diagnosis. These patients had a normal preoperative neurological

assessment but poorer-than-expected mean scores on cognitive and psychomotor

development were found at 1-year follow-up.22 At 5-year follow-up, 35% of patients

demonstrated clinically significant difficulties in cognition, academic achievement, or

executive functioning.23 It was hypothesized that these adolescents would experience

poorer HRQoL and display poorer performance than same-age peers across a range of

tests.

METHOD

Participants

Families of 43 adolescents who had undergone cardiopulmonary bypass surgery for

CHD between 1999 and 2001 were contacted. Adolescents and caregivers attended the

Lady Cilento Children’s Hospital, Brisbane, Australia, for assessment. Informed

consent was obtained from caregivers and participants. Demographic data were

collected. Participants underwent a medical review, and genetic screening was

performed with consent.

Measures

A psychometric assessment protocol was developed to provide a broad view of

outcomes for adolescents with CHD. Consideration was given to assessments used in

key studies worldwide, and, where possible, equivalent measures were used to better

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contribute to the international community’s knowledge of CHD outcomes.

Assessments were: the Wechsler Intelligence Scale for Children, Fourth Edition;

Wechsler Adult Intelligence Scale, Fourth Edition; Wechsler Individual Achievement

Test, Third Edition; Wide Range Assessment of Memory and Learning, Second

Edition; California Verbal Learning Test – Children’s Version; Test of Everyday

Attention for Children; Behaviour Rating Inventory of Executive Function; Conners,

Third Edition; Adaptive Behavior Assessment System, Second Edition; Behavior

Assessment System for Children, Second Edition; Rey–Osterrieth Complex Figure; and

Grooved Pegboard. Two psychologists administered assessments under standardized

conditions. The Pediatric Quality of Life Inventory 4.0 Generic Core Scales and disease

specific Pediatric Quality of Life Inventory 3.0 Cardiac module were used to measure

HRQoL by self- and parent-proxy-report,3 and were administered by the research nurse

coordinator before psychological and medical assessment.

Statistical methods

Data analysis was performed using SPSS Statistics Version 23 (IBM, Armonk, NY,

USA). Variables were screened for missing data, outliers, and normality. Outliers were

identified on the Rey–Osterrieth Complex Figure and removed. In several scales, data

were missing due to non-completion. One participant with hearing loss was

administered a modified protocol to prevent inclusion of results associated with deficits

in hearing. Several other outcomes were for slightly reduced sample numbers due to

failure to complete assessments. Numbers in tables indicate the affected scales.

For normal distributions, one-sample t-tests were used to compare participants’

results with the standardization samples on which individual tests were normed. Some

results for the Wechsler Individual Achievement Test, Third Edition, Adaptive

Behavior Assessment System, Second Edition, Behaviour Rating Inventory of

Executive Function, Behavior Assessment System for Children, Second Edition, and

Conners, Third Edition scales or subtests did not meet assumptions of normality due to

skew, which transformation could not resolve. Where magnitude of significance and

effect size were equivalent for parametric and non-parametric tests (one-sample

Wilcoxon signed-rank test), analyses were assumed to be robust to violations of

normality and t-test results were reported. Otherwise, the Wilcoxon signed-rank test

was reported. For the Pediatric Quality of Life Inventory scale, self-report scores were

compared with population norms and parent-proxy-report scores using t-tests. To

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account for multiple comparisons, a Benjamini–Hochberg correction was performed.24

As a result, tests were considered significant at an adjusted p-value of less than or equal

to 0.01.

Ethics

Ethics approval was granted by the Queensland University of Technology Human

Research Ethics Committee (Approval Number: 1300000716) and the Mater Health

Services Human Research Ethics Committee (Reference Number: HRED/13/MHS/28).

RESULTS

Of 43 families contacted, one family had moved overseas, eight could not be located,

six refused, four failed to attend appointments, and two were excluded owing to

syndromes diagnosed postoperatively (CHARGE syndrome [coloboma of the eye,

heart defects, atresia of the choanae, retardation of growth and/or development, genital

and/or urinary abnormalities, ear abnormalities and deafness] and 22q11 deletion). As

a result, 21 participants (10 males, 11 females) were recruited, ranging in age from 14

to 17 years (mean 15y 4.8mo, SD 8.4mo). Statistical analysis was performed on

available data to compare the lost-to-follow-up group with the study participants

(independent-sample t-test for continuous data, and a 2 test of contingencies for

categorical variables). No significant differences were found between the groups on

any of the available variables: diagnosis, sex, gestational age, circulatory arrest status,

geographical region, age at repair, weight at repair, bypass time, or Bayley Scales of

Infant Development mental and motor scores at 1 year of age. The results of these

analyses are available in Table SI (online supporting information).

Of the 21 participants in this study, 19 were white, one reported an Indigenous

Australian background, and one reported an unspecified ethnicity. Ten participants

were from metropolitan areas and 11 from regional and remote locations.

Seven participants had a diagnosis of transposition of the great arteries, five had

a diagnosis of tetralogy of Fallot, five had a ventricular septal defect, one participant

had total anomalous pulmonary venous drainage, one had complex functionally single

ventricle, one had congenitally corrected transposition of the great arteries, and one had

valvar pulmonary stenosis (Table I). Twenty participants had biventricular repairs, and

nine had required subsequent surgery, with all participants at least 6 months after their

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most recent open-heart surgery (Table I). All participants had normal oxygen

saturations and were classified as New York Heart Association class I, reporting peak

physical activity ranging from sedentary to competitive sport. A deletion was detected

on 8q22.3 following single nucleotide polymorphism array analysis in one patient.

Table I also provides information on birthweight and gestational age for this sample.

Cognition and academic achievement

Participants’ overall cognitive ability, as measured by Full-scale IQ, was lower than,

but did not differ significantly from, the general population (p=0.06). Composite score

means for Verbal Comprehension, Perceptual Reasoning, Working Memory, and

Processing Speed were lower than the general population, although only Processing

Speed remained significant after applying the adjusted significance test for multiple

comparisons (p=0.009; Table II). For measures of academic achievement, participants

scored significantly below expected for Numerical Operations (p=0.002; Table II).

Memory, attention, and executive function

Participants’ Story and Design Memory scores from the Wide Range Assessment of

Memory and Learning, Second Edition and scores on a measure of verbal learning and

memory from the California Verbal Learning Test – Children’s Version were not

significantly different from those of the standardization sample after adjusting for

multiple comparisons (Table II).

Participants scored significantly below expected on a measure of sustained

attention and response inhibition (Walk Don’t Walk; p=0.006). Scores were elevated

compared with population norms (indicating greater difficulties) on adolescent self-

report for the Behaviour Rating Inventory of Executive Function measure of executive

function, although there were no significant differences after adjusting for multiple

comparisons (Table II).

Visual perception, visual-construction, and motor abilities

Participants scored below expected on a one-sample t-test compared with published age

norms for the Rey–Osterrieth Complex Figure copy task (p=0.010). Strategies

employed by participants to complete the copy task were noted, and it was observed

that 17 participants (81%) used a piecemeal approach, while only four (19%) used a

configurational approach. Participants’ Grooved Pegboard scores, a measure of visual-

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motor coordination, were below expected for the dominant hand task when compared

with published norms (p=0.004) (Table II).

Adaptive behaviour, social-emotional, and behavioural outcomes

No significant differences were found for adaptive behaviour subscales or indices

(Table III). According to Conners, Third Edition self-report data, participants in this

study reported more learning problems than the standardization sample (p=0.009).

They also reported less-elevated scores on the Family Relations Index, indicating better

family relations than the standardization sample (p<0.001).

HRQoL

In the Generic Core Scales, total self-reported HRQoL (p=0.003) and physical

(p=0.004) and psychological (p=0.003) summary scores were significantly lower than

population norms, although no significant difference in social functioning was reported.

On parent-report, psychological summary scores were significantly lower (Table IV).

In the disease-specific module, lowest self-reported scores were in the areas of

cognitive problems (mean 60.05) and perceived physical appearance (mean 62.08).

Self- and parent-proxy reporting was largely consistent across both generic and disease-

specific scales, except for social functioning (p=0.01).

DISCUSSION

This study aimed to increase understanding of neurodevelopmental outcomes and

HRQoL in adolescence associated with surgery for CHD in infancy, through a

combination of psychometric assessment measures and parent and adolescent

questionnaires. As a group, participants displayed difficulties in a number of areas,

including cognitive functioning, academic achievement, attention, organization,

emotional and behavioural functioning, and HRQoL. These results support findings

reported in the wider literature on outcomes for individuals with CHD.

Mean overall cognitive ability was within the average range, in line with other

research on cognition in children with CHD,25 although some studies found mean IQ

scores for children with CHD to be slightly below the population mean.26 This also

corresponds with outcomes reported from two earlier studies of this cohort, where

measures were within the average range,22,23 although at the 5-year follow-up

assessment, 35% of children displayed significant difficulties in at least one area. The

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clinical implication of these findings is that without comprehensive screening, potential

neuropsychological impairment may be missed.26

Group performance was below expected on Numerical Operations, and,

according to self-report, participants scored significantly higher than expected for

learning problems. Other studies have also found CHD to be associated with poorer

academic achievement,1,18 although it is noted that results for this group on Spelling,

Word Reading, and Pseudoword Decoding were within the expected range. Some

research supports the suggestion that for individuals with CHD, lower-level skills can

be unaffected, but difficulties emerge when undertaking more complex tasks. Bellinger

and Newburger assessed children with CHD at 8 years of age,14 and found that while

single-word reading was at age level, children performed below expected on reading

comprehension tasks. They also found while children understood basic maths concepts,

they were unable to apply them to solve specific problems. In the present study, the

subtest Numerical Operations required participants to not only apply concepts to solve

problems, but also initiate, plan, and organize their work, and it may be that deficits of

executive function contributed to poorer results.

Differences in areas of ability associated with cognitive flexibility were found,

with scores on measures of attention and planning and organization lower than

expected. Prior research has also found cognitive flexibility to be a key area of

impairment, with 16-year-olds with complex CHD reported to have difficulty switching

between sequences or categories, when assessed via standardized laboratory

assessments.14 Another study of individuals aged 10 to 19 years with CHD also

identified problems with cognitive flexibility and shifting, consistent across self- and

parent-report and also laboratory tasks, although relaxed cut-off scores were used.6

Cognitive flexibility is thought to develop later than other executive functions

and increases capacity for perspective-taking and adjustment to changes.6,27 As such, it

contributes to both academic achievement and social well-being.1,6 The later emergence

of cognitive flexibility as an executive function may be associated with the pattern

found in individuals with CHD, where difficulties become more apparent in late

childhood.14

Difficulties with metacognition, which encompasses the ability to initiate, plan,

monitor, and sustain working memory to solve problems, have also been found to be

more prevalent in children with CHD.1 Gerstle et al. reported results showing that

metacognition is impaired in children with CHD from 8 to 16 years.1 They found

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impairment was correlated with age, with the prevalence of metacognitive impairment

increasing throughout adolescence. The authors suggested that children with CHD may

experience delayed metacognitive development, and this is potentially a factor in the

emergence of difficulties in adolescence.1

Results also indicated poorer visual-spatial ability in participants, when

compared to a normative sample. In addition, the Rey–Osterrieth Complex Figure

task provided information about organization, as well as visual-spatial ability.

Qualitative observations suggest participants used a less integrated strategy when

copying the figure, with 81% using a piecemeal approach. Research suggests that, in

the general population, most children stop using a piecemeal approach by 13 years of

age.28 Similar results have been reported in 8-year-old children and adolescents with

CHD.9,29 Both earlier studies found that participants’ poor organization when

approaching the task negatively affected performance.

Differences emerged between self- and parent-report on measures of behaviour

and emotional well-being. While parents tended to report behaviour as more favourable

than the standardization sample, self-report indicated participants considered they

experienced more learning problems than the general population. Adolescents did,

however, report more positive family relations than the standardization sample.

These results contradict Bellinger and Newburger’s research on

neurodevelopmental outcomes,14 which found both parents and adolescents reported

significantly more attention-deficit/hyperactivity disorder-associated behaviours, while

adolescents reported more aggression and conduct disorder. However, all participants

in the cited study were diagnosed with transposition of the great arteries, while only

33% of our participants had this diagnosis, and while this study’s small sample size

precluded statistical analysis of specific diagnostic factors, they may influence

outcomes. Additionally, it may be important to consider cultural differences in parent

reporting of child behaviour, as research has previously found differences in reporting

between the US and Australia.30 Contradictions in the literature around behavioural and

emotional functioning for children with CHD have been noted previously,5 and

highlight the benefits of using a range of measures where possible, rather than single

informant report, as well as being aware of inclusion and exclusion criteria and cultural

norms.

Adolescents in this study reported significantly decreased HRQoL when

compared with population norms, consistent with other studies of children and

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adolescents with moderate, biventricular disease.31 Lower scores in school and

cognitive functioning are consistent with deficits found in the neuropsychological

assessment. Further exploration of self-identified social competence and impact of

potential parental underestimation of their child’s social functioning may be beneficial.

While a range of differences have been found between this group of adolescents

and the normative population, on the individual level, participants at times performed

within or above the expected ranges for their age. While adolescents with CHD may be

at higher risk for impairment, individual outcomes can be variable.

Study limitations and strengths

Although participants were drawn from a prospective cohort study of 47 infants

originally, participation varied across the three follow-up studies, with longitudinal data

only available for 14 participants. This limited the study’s ability to investigate

potential predictive factors in adolescent outcomes for CHD. It is also possible that a

range of factors, such as parental concern, influenced participation, leading to a

subgroup not representative of the population overall.

The total number of participants (n=21) in this phase of the research is also a

limitation, as the sample size did not allow sufficient power to examine correlations

among variables, including potential differences in outcome with regard to diagnostic

category. Nevertheless, a retention rate of close to 50% over a period of 17 years is

notable. Longitudinal studies are relatively rare, yet have the potential to provide data

of immense value.

Another limitation of the current research is that not all participants agreed to

genetic testing. An abnormality was detected in one of 15 children tested. Other

participants in the cohort, although phenotypically normal, may have unidentified

chromosomal abnormalities, which may adversely affect neurodevelopmental and

HRQoL findings.32

Despite these limitations, the current study has some notable strengths. The

assessment battery was comprehensive, covering all domains recommended for

screening individuals with CHD by the American Heart Association.13 This made it

possible to identify specific deficits, such as with processing speed and attention, even

though broader constructs such as Full-scale IQ were within the average range. It also

included measures of social competence, emotional well-being, and family relations,

domains in which executive function may contribute to more positive outcomes but

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which prior studies have not always incorporated.4,6 Finally, the study used both task-

based measures and self-report for many domains, as relying on only one type of

assessment tool may produce seemingly contradictory findings.5

CONCLUSION

This research contributes to the small body of knowledge about neurodevelopmental

outcomes for adolescents with CHD, a group that, to date, has received considerably

less attention internationally than younger children.17 Increased understanding is likely

to have important implications for policies and practices regarding neurodevelopmental

screening programmes and early interventions for children born with CHD and their

families. The Queensland Paediatric Cardiac Service’s neurodevelopmental follow-up

program (CHD LIFE) has undertaken a body of work to develop a state-wide

developmental long-term care pathway informed by the American Heart Association’s

recommendations for neurodevelopmental evaluation and management in this

population. Challenges lie in ensuring children and adolescents in the schooling years

can access appropriate surveillance and intervention. This project’s outcomes provide

evidence supporting the ongoing need for this service in our local population.

Future research can benefit from the growing body of knowledge through

clinical trials of interventions targeting identified difficulties. With executive function

emerging as a key area of concern, there are many possible approaches to explore, from

physical activity and computerized programs targeting specific skills,15 through to

family-focused interventions.33 Ongoing follow-up of individuals with CHD as they

enter adulthood will be important, to further understand the impact of CHD across the

lifespan.

ACKNOWLEDGEMENTS

This study was supported by funding from HeartKids Australia Research Grants-in-

Aid. We would like to acknowledge the support of Samantha Archer (Queensland

University of Technology), Robert Ware (Griffith University), Janelle Johnson

(Queensland Paediatric Cardiac Service), and Jessica Suna (Queensland Paediatric

Cardiac Service). We also gratefully acknowledge the support of the participants and

their families who volunteered their time to take part in this study. The authors have

stated that they had no interests which might be perceived as posing a conflict or bias.

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Supporting information

The following additional material may be found online:

Table SI: Comparison of loss to follow-up with current study participants by

diagnostic, clinical, and demographic variables

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Table I: Diagnostic and surgical characteristics of participants (n=21)

Variable n (%)

Diagnosis

Transposition of the great arteries 7 (33)

Tetralogy of Fallot 5 (24)

Ventricular septal defect 5 (24)

Total anomalous pulmonary venous drainage 1 (5)

Complex functionally single ventricle 1 (5)

Congenitally corrected transposition of the great arteries 1 (5)

Valvar pulmonary stenosis 1 (5)

Sex

Female 11 (52)

Male 10 (48)

Gestational age (wk)

<37 3 (14)

37–40 16 (76)

>40 2 (10)

Birthweight (kg)a

<2.5 2 (10)

2.5–4.0 15 (71)

>4.0 2 (10)

Age at repair (d)

<10 3 (14)

10–30 8 (38)

30–60 5 (24)

>60 5 (24)

Congenital abnormalities

Yes 1 (5)

No 20 (95)

Total number of cardiac surgeries

One 12 (57)

Two 7 (33)

Three or more 2 (10)

Total bypass time (min)

<100 5 (24)

18

100–200 10 (48)

>200 6 (29)

aBirthweight data not available for two participants.

Table II: Cognition, academic achievement, and executive function in adolescents

with congenital heart disease

Mean SD Score n p 9Intelligence (mean 100, SD 15)

Full-scale IQ 94.30 12.73 64–111 20 0.060 –

Verbal comprehension 96.35 13.18 73–121 21 0.231 –

Perceptual reasoning 97.85 12.75 67–115 21 0.281 –

Working memory 94.00 11.98 62–110 21 0.023 –

Processing speed 93.25 11.51 70–118 20 0.009a –

Academic achievement (mean

100, SD 15)

Maths problem solving 95.25 14.37 72–125 21 0.079 –

Spelling 102.45 18.09 55–132 20 0.552 –

Word readingb 104 20 0.058

Pseudoword decodingb 104 20 0.184

Numerical operationsb 81 21 0.002a

Memory (mean 10, SD 3)

Story memory 10.5 2.67 5–16 20 0.412 –

Design memory 9.55 2.19 6–13 21 0.226 –

Verbal learning (mean 50, SD 10)

Total recall performance 44.37 11.13 20–61 19 0.041 –

Attention (mean 10, SD 3)

Creature counting –

switching

10.39 3.13 5–14 18 0.605 –

Walk don’t walk –

response inhibition

7.11 3.94 1–15 18 0.006a –

Opposite world –

control/switching

8.50 2.38 3–12 18 0.016 –

BRIEF Self-Report (mean 50, SD

10)

Behavioural Regulation

Indexc

55.05 12.85 36–79 20 0.095 –

Working Memoryc 55.90 12.94 35–75 20 0.056 –

20

Metacognition Indexc 56.00 12.23 35–75 20 0.041 0

Global Executive

Compositec

56.05 13.21 35–79 20 0.055 –

BRIEF Parent (mean 50, SD 10)

Behavioural Regulation

Indexc

51.29 10.13 38–74 21 0.567 –

Metacognition Indexc 53.90 12.64 35–77 21 0.172 –

Global Executive

Compositec

53.14 11.45 35–71 21 0.223 –

Rey–Osterrieth Complex Figure

(mean 33.6, SD 2.98)

Copy 30.35 5.07 15–35 20 0.010a –

Grooved Pegboard

Dominant Hand (mean 66.05, SD

10.4)c

72.29 8.83 54–86 21 0.004a 2

Non-dominant hand (mean 70.5,

SD 11.1)c

73.67 8.39 61–89 21 0.099 –

Means and standard deviations (SD) of standardization samples are included beside

each measure in the table. CI, confidence interval; BRIEF, Behaviour Rating

Inventory of Executive Function. aSignificant at Benjamini–Hochberg-adjusted

significance level of p<0.01. bWilcoxon signed-rank test used, median reported.

cHigher scores indicate poorer outcomes.

21

Table III: Measures of adaptive behaviour, behaviour, and social and emotional well-

being in adolescents with congenital heart disease

Mean/median SD Score (range) n p

ABAS-II domains (mean 100, SD 15) GACa 94.32 19.39 64 to >120 19 0.218Conceptuala 100.05 16.58 72 to >120 19 0.989Sociala 95.95 15.03 66 to >120 19 0.255Practicala 91.26 18.78 58 to >120 19 0.058

Conners-3 Self-Report (mean 50, SD

10)

Inattention 54.95 10.97 40–74 20 0.058Hyperactivity/Impulsivity 53.10 11.10 40–83 20 0.227Learning Problems 56.95 10.63 40–78 20 0.009b

Defiance/Aggression 48.75 7.56 40–70 20 0.469Family relations 45.75 4.44 40–54 20 <0.001DSM-IV ADHD Inattentive 55.05 11.11 40–78 20 0.056DSM-IV ADHD 52.05 11.22 40–85 20 0.424Conduct Disorder 48.35 8.16 40–77 20 0.377Oppositional Defiant Disorder 49.25 5.84 40–59 20 0.572

Conners-3 Parent (mean 50, SD 10) Inattentionc 55 40–80 21 0.076

Hyperactivity/Impulsivity 51.00 9.18 41–67 21 0.623Learning Problems 56.43 14.48 40–90 21 0.055Executive Function 54.33 12.92 40–76 21 0.140Defiance/Aggression 49.76 7.11 43–64 21 0.880Peer Relations 57.76 17.45 42–90 21 0.055Global Index Total 54.24 10.43 40–72 21 0.077DSM-IV ADHD Inattentive 54.05 11.98 40–79 21 0.137DSM-IV ADHD Hyperactive 51.90 10.34 41–73 21 0.409Conduct Disorder 47.67 4.27 43–57 21 0.021

BASC-2 Self Report (mean 50, SD School Problems Composite 52.90 12.09 37–75 20 0.297Internalising Problems 53.60 12.50 38–82 20 0.213Inattention Hyperactivity 53.60 12.83 32–75 20 0.225Personal Adjustment 46.30 12.94 16–61 20 0.216Emotional Symptoms Index 54.95 15.15 35–87 20 0.160

BASC-2 Parent Report (mean 50, SD Externalising Problems 46.14 7.00 37–58 21 0.020Internalising Problems 56.14 18.46 36–101 21 0.143Behavioural Symptoms Index 51.00 12.46 37–86 21 0.717Adaptive Skillsa 47.86 11.13 29–72 21 0.388

Note. Means and standard deviations (SD) of standardization samples are included

beside each measure in the table. CI, confidence interval; ABAS-II, Adaptive

Behavior Assessment System, Second Edition; GAC, General Adaptive Composite;

Conners-3, Conners, Third Edition; ADHD, attention-deficit–hyperactivity disorder;

22

BASC-2, Behavior Assessment System for Children, Second Edition. aLower scores

indicate poorer outcomes. bSignificant at Benjamini–Hochberg adjusted significance

level of p<0.01. cWilcoxon signed-rank test used, median, z score, and r effect size

reported.

23

Table IV: Core scales. Study participants vs population norms

Scale CHD sample Control samplea

n Mean SD n Mean SD p 95% CI

Self-report

Total score 20 74.94 14.10 1066 85.49 12.04 0.003b –17.14 to –3.94 Physical health 20 79.38 13.19 1064 88.79 13.60 0.005b –15.59 to –3.24 Psychosocial health 20 70.52 17.55 1064 83.74 13.38 0.003b –21.43 to –5.00 Emotional function 20 64.75 21.79 1066 80.82 17.75 0.004b –26.27 to –5.87 Social function 20 79.50 25.02 1061 89.27 14.50 0.097 –21.48 to 1.94 School function 20 67.50 16.50 1060 81.20 16.75 0.001b –21.42 to –5.98 Proxy report

Total score 21 74.79 15.48 1281 81.75 15.72 0.053 –14.00 to 0.09 Physical health 21 81.02 15.70 1279 83.87 20.13 0.415 –10.00 to 4.29 Psychosocial health 21 68.57 18.15 1283 80.55 15.82 0.007b –20.24 to 3.72 Emotional function 21 65.95 21.60 1278 80.56 17.81 0.006b –24.44 to –4.78 Social function 21 70.48 23.29 1283 84.23 19.30 0.014 –24.35 to –3.15 School function 21 69.29 22.98 1274 76.96 20.02 0.142 –18.14 to 2.79

Higher scores equal better health-related quality of life. aFrom Pediatric Quality of Life

Inventory Healthy Children Database. bSignificant at Benjamini–Hochberg-adjusted

significance level of p<0.01. CHD, congenital heart disease; SD, standard deviation.