Skip to main content Accessibility help

Executive function deficits in congenital heart disease: why is intervention important?

  • Johanna Calderon (a1) (a2) and David C. Bellinger (a1) (a3)


It is widely recognised that children with congenital heart disease (CHD) are at high risk for neurodevelopmental impairments including attention deficit hyperactivity disorder and autism spectrum disorder symptoms. Executive function impairments are one of the most prominent neurodevelopmental features associated with CHD. These deficits can have widespread debilitating repercussions in children’s neurocognitive, behavioural, and psycho-social development. There is a crucial gap in research regarding the efficacy of preventive or treatment strategies for these important cognitive morbidities. Executive functions are complex neurocognitive skills highly amenable to improvement. Evidence-based interventions have shown promising results in other paediatric populations, strongly suggesting that they might also benefit the growing population of children with CHD. In this review, we summarise the available data on executive function impairments in children and adolescents with CHD. We underline the important co-morbidity of executive dysfunction with other cognitive and psychiatric issues in CHD, which raises awareness of the crucial need to prevent or at least mitigate these deficits. Finally, we summarise future avenues for research in terms of interventions that may help reduce executive function impairments in youth with CHD.

  • View HTML
    • Send article to Kindle

      To send this article to your Kindle, first ensure is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

      Note you can select to send to either the or variations. ‘’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

      Find out more about the Kindle Personal Document Service.

      Executive function deficits in congenital heart disease: why is intervention important?
      Available formats

      Send article to Dropbox

      To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

      Executive function deficits in congenital heart disease: why is intervention important?
      Available formats

      Send article to Google Drive

      To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

      Executive function deficits in congenital heart disease: why is intervention important?
      Available formats


Corresponding author

Correspondence to: J. Calderon, PhD, Boston Children’s Hospital, 300 Longwood Avenue, Boston, MA 02115, United States of America. Tel: +857 218 5063; Fax: +617 730 0618; E-mail:


Hide All
1. Marino, BS, Lipkin, PH, Newburger, JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management a scientific statement from the American Heart Association. Circulation 2012; 126: 11431172.
2. Bellinger, DC, Newburger, JW, Wypij, D, Kuban, KCK, duPlesssis, AJ, Rappaport, LA. Behaviour at eight years in children with surgically corrected transposition: the Boston Circulatory Arrest Trial. Cardiol Young 2009; 19: 8697.
3. Shillingford, AJ, Glanzman, MM, Ittenbach, RF, Clancy, RR, Gaynor, JW, Wernovsky, G. Inattention, hyperactivity, and school performance in a population of school-age children with complex congenital heart disease. Pediatrics 2008; 12: 759767.
4. Sistino, JJ, Atz, AM, Simpson, KN, Ellis, C, Ikonomidis, JS, Bradley, SM. The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair. Cardiol Young 2015; 25: 663669.
5. Hansen, E, Poole, TA, Nguyen, V, et al. Prevalence of ADHD symptoms in patients with congenital heart disease. Pediatr Int 2012; 54: 838843.
6. Bellinger, DC, Newburger, JW. Neuropsychological, psychosocial, and quality-of-life outcomes in children and adolescents with congenital heart disease. Prog Pediatr Cardiol 2010; 29: 8792.
7. Bellinger, DC, Bernstein, JH, Kirkwood, MW, Rappaport, LA, Newburger, JW. Visual-spatial skills in children after open-heart surgery. J Dev Behav Pediatr 2003; 24: 169179.
8. Bellinger, DC, Wypij, D, Rivkin, MJ, et al. Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure: neuropsychological assessment and structural brain imaging. Circulation 2011; 124: 13611369.
9. Calderon, J, Bonnet, D, Courtin, C, Concordet, S, Plumet, MH, Angeard, N. Executive function and theory of mind in school-aged children after neonatal corrective cardiac surgery for transposition of the great arteries. Dev Med Child Neurol 2010; 52: 11391144.
10. Calderon, J, Angeard, N, Moutier, S, Plumet, MH, Jambaqué, I, Bonnet, D. Impact of prenatal diagnosis on neurocognitive outcomes in children with transposition of the great arteries. J Pediatr 2012; 161: 9498.
11. Calderon, J, Angeard, N, Pinabiaux, C, Bonnet, D, Jambaqué, I. Facial expression recognition and emotion understanding in children after neonatal open-heart surgery for transposition of the great arteries. Dev Med Child Neurol 2014; 56: 564571.
12. Bellinger, DC, Wypij, D, duPlessis, AJ, et al. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003; 126: 13851396.
13. Bellinger, DC, Rivkin, MJ, DeMaso, D, et al. Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging. Cardiol Young 2015; 25: 338347.
14. Calderon, J, Jambaqué, I, Bonnet, D, Angeard, N. Executive functions development in 5- to 7-year-old children with transposition of the great arteries: a longitudinal study. Dev Neuropsychol 2014; 39: 365384.
15. Miatton, M, De Wolf, D, François, K, Thiery, E, Vingerhoets, G. Neuropsychological performance in school-aged children with surgically corrected congenital heart disease. J Pediatr 2007; 151: 7378.
16. Hövels-Gürich, HH, Konrad, K, Skorzenski, D, Herpertz-Dahlmann, B, Messmer, BJ, Seghaye, MC. Attentional dysfunction in children after corrective cardiac surgery in infancy. Ann Thorac Surg 2007; 83: 14251430.
17. Gaynor, JW, Gerdes, M, Nord, AS, et al. Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy? J Thorac Cardiovasc Surg 2010; 140: 12301237.
18. Calderon, J, Bonnet, D, Pinabiaux, C, Jambaqué, I, Angeard, N. Use of early remedial services in children with transposition of the great arteries. J Pediatr 2013; 163: 11051110.
19. Neal, AE, Stopp, C, Wypij, D, et al. Predictors of health-related quality of life in adolescents with tetralogy of Fallot. J Pediatr 2015; 166: 132138.
20. Diamond, A. Executive functions. Annu Rev Psychol 2013; 64: 135168.
21. Lehto, JE, Juujarvi, P, Kooistra, L, Pulkkinen, L. Dimensions of executive functioning: evidence from children. Br J Dev Psychol 2003; 21: 59.
22. Diamond, A, Barnett, WS, Thomas, J, Munro, S. Preschool program improves cognitive control. Science 2007; 318: 13871388.
23. Diamond, A. Normal development of prefrontal cortex from birth to young adulthood: cognitive functions, anatomy, and biochemistry. In: Stuss DT, Knight RT (eds). Principles of Frontal Lobe Function. Oxford University Press, New York, NY, USA, 2002: 466503.
24. Carlson, SM. Developmentally sensitive measures of executive function in preschool children. Dev Neuropsychol 2005; 28: 595616.
25. Hughes, C, Ensor, R. Executive function and theory of mind: predictive relations from ages 2 to 4. Dev Psychol 2007; 43: 14471459.
26. Davidson, MC, Amso, D, Anderson, LC, Diamond, A. Development of cognitive control and executive functions from 4 to 13 years: evidence from manipulations of memory, inhibition, and task switching. Neuropsychologia 2006; 44: 20372078.
27. Luna, B, Garver, KE, Urban, TA, Lazar, NA, Sweeney, JA. Maturation of cognitive processes from late childhood to adulthood. Child Dev 2004; 75: 13571372.
28. Durston, S, Davidson, MC, Tottenham, N, et al. A shift from diffuse to focal cortical activity with development. Dev Sci 2006; 9: 18.
29. Liston, C, Watts, R, Tottenham, N, et al. Frontostriatal microstructure modulates efficient recruitment of cognitive control. Cereb Cortex 2006; 16: 553560.
30. Casey, BJ, Galvan, A, Hare, TA. Changes in cerebral functional organization during cognitive development. Curr Opin Neurobiol 2005; 15: 239244.
31. Barkley, RA. Distinguishing sluggish cognitive tempo from ADHD in children and adolescents: executive functioning, impairment, and comorbidity. J Clin Child Adolesc Psychol 2013; 42: 161173.
32. Woodward, LJ, Clark, CAC, Bora, S, Inder, TE. Neonatal white matter abnormalities an important predictor of neurocognitive outcome for very preterm children. PLoS One 2012; 7: 51879.
33. Blair, C, Razza, RP. Relating effortful control, executive function, and false belief understanding to emerging math and literacy ability in kindergarten. Child Dev 2007; 78: 647663.
34. Carlson, SM, Moses, LJ, Breton, C. How specific is the relation between executive function and theory of mind? Contributions of inhibitory control and working memory. Infant Child Dev 2002; 11: 7392.
35. Pellicano, E. Individual differences in executive function and central coherence predict developmental changes in theory of mind in autism. Dev Psychol 2010; 46: 530544.
36. Taylor Tavares, JV, Clark, L, Cannon, DM, Erickson, K, Drevets, WC, Sahakian, BJ. Distinct profiles of neurocognitive function in unmedicated unipolar depression and bipolar II depression. Biol Psychiatry 2007; 62: 917924.
37. Baler, RD, Volkow, ND. Drug addiction: the neurobiology of disrupted self-control. Trends Mol Med 2006; 12: 559566.
38. Miller, AL, Lee, HJ, Lumeng, JC. Obesity-associated biomarkers and executive function in children. Pediatr Res 2015; 77: 143147.
39. Bailey, CE. Cognitive accuracy and intelligent executive function in the brain and in business. Ann N Y Acad Sci 2007; 1118: 122141.
40. Cassidy, AR, White, MT, DeMaso, DR, Newburger, JW, Bellinger, DC. Executive function in children and adolescents with critical cyanotic congenital heart disease. J Int Neuropsychol Soc 2014; 9: 116.
41. Von Rhein, M, Buchmann, A, Hagmann, C, et al. Brain volumes predict neurodevelopment in adolescents after surgery for congenital heart disease. Brain 2014; 137: 268276.
42. Von Rhein, M, Kugler, J, Liamlahi, R, Knirsch, W, Latal, B, Kaufmann, L. Persistence of visuo-constructional and executive deficits in adolescents after open-heart surgery. Res Dev Disabil 2014; 36: 303310.
43. Schaefer, C, von Rhein, M, Knirsch, W, et al. Neurodevelopmental outcome, psychological adjustment, and quality of life in adolescents with congenital heart disease. Dev Med Child Neurol 2013; 55: 11431149.
44. DeMaso, DR, Labella, M, Taylor, GA, et al. Psychiatric disorders and function in adolescents with d-transposition of the great arteries. J Pediatr 2014; 165: 760766.
45. Freitas, IR, Castro, M, Sarmento, SL, et al. A cohort study on psychosocial adjustment and psychopathology in adolescents and young adults with congenital heart disease. BMJ Open 2013; 3: 18.
46. Brock, LL, Brock, CD, Thiedke, CC. Executive function and medical non-adherence: a different perspective. Int J Psychiatry Med 2011; 42: 105115.
47. Iversen, K, Vejlstrup, NG, Sondergaard, L, Nielsen, OW. Screening of adults with congenital cardiac disease lost for follow-up. Cardiol Young 2007; 17: 601608.
48. Levy, S, Katusic, SK, Colligan, RC, et al. Childhood ADHD and risk for substance dependence in adulthood: a longitudinal, population-based study. PLoS One 2014; 9: 105640.
49. Albers, EL, Bichell, DP, McLaughlin, B. New approaches to neuroprotection in infant heart surgery. Pediatr Res 2010; 68: 19.
50. Marino, BS. New concepts in predicting, evaluating, and managing neurodevelopmental outcomes in children with congenital heart disease. Curr Opin Pediatr 2013: 29.
51. McCusker, CG, Doherty, NN, Molloy, B, et al. A randomized controlled trial of interventions to promote adjustment in children with congenital heart disease entering school and their families. J Pediatr Psychol 2012; 37: 10891103.
52. Söderqvist, S, Bergman Nutley, S, Peyrard-Janvid, M, et al. Dopamine, working memory, and training induced plasticity: implications for developmental research. Dev Psychol 2012; 48: 836843.
53. Bergman Nutley, S, Söderqvist, S, Bryde, S, Thorell, LB, Humphreys, K, Klingberg, T. Gains in fluid intelligence after training non-verbal reasoning in 4-year-old children: a controlled, randomized study. Dev Sci 2011; 14: 591601.
54. Rueda, MR, Checa, P, Cómbita, LM. Enhanced efficiency of the executive attention network after training in preschool children: immediate changes and effects after two months. Dev Cogn Neurosci 2012; 15: 192204.
55. Thorell, LB, Lindqvist, S, Bergman Nutley, S, Bohlin, G, Klingberg, T. Training and transfer effects of executive functions in preschool children. Dev Sci 2009; 12: 106113.
56. Holmes, J, Gathercole, SE, Dunning, DL. Adaptive training leads to sustained enhancement of poor working memory in children. Dev Sci 2009; 12: 915.
57. Klingberg, T, Forssberg, H, Westerberg, H. Training of working memory in children with ADHD. J Clin Exp Neuropsychol 2002; 24: 781791.
58. Klingberg, T, Fernell, E, Olesen, PJ, et al. Computerized training of working memory in children with ADHD–a randomized, controlled trial. J Am Acad Child Adolesc Psychiatry 2005; 44: 177186.
59. Grunewaldt, KH, Løhaugen, GCC, Austeng, D, Brubakk, AM, Skranes, J. Working memory training improves cognitive function in VLBW preschoolers. Pediatrics 2013; 131: 747754.
60. Løhaugen, GCC, Antonsen, I, Håberg, A, et al. Computerized working memory training improves function in adolescents born at extremely low birth weight. J Pediatr 2011; 158: 555561.
61. Diamond, A, Lee, K. Interventions shown to aid executive function development in children 4 to 12 years old. Science 2011; 333: 959964.
62. Kronenberger, WG, Pisoni, DB, Henning, SC, Colson, BG, Hazzard, LM. Working memory training for children with cochlear implants: a pilot study. J Speech Lang Hear Res 2011; 54: 11821196.
63. Olesen, PJ, Nagy, Z, Westerberg, H, Klingberg, T. Combined analysis of DTI and fMRI data reveals a joint maturation of white and grey matter in a fronto-parietal network. Brain Res Cogn Brain Res 2003; 18: 4857.
64. Astle, DE, Barnes, JJ, Baker, K, Colclough, GL, Woolrich, MW. Cognitive training enhances intrinsic brain connectivity in childhood. J Neurosci 2015; 35: 62776283.
65. Green, CT, Long, DL, Green, D, et al. Will working memory training generalize to improve off-task behavior in children with attention-deficit/hyperactivity disorder. Neurotherapeutics 2012; 9: 639648.
66. Flook, L, Goldberg, SB, Pinger, L, Davidson, RJ. Promoting prosocial behavior and self-regulatory skills in preschool children through a mindfulness-based kindness curriculum. Dev Psychol 2015; 51: 4451.
67. Tang, YY, Yang, L, Leve, LD, Harold, GT. Improving executive function and its neurobiological mechanisms through a mindfulness-based intervention: advances within the field of developmental neuroscience. Child Dev Perspect 2012; 6: 361366.
68. Dulfer, K, Duppen, N, Kuipers, IM, et al. Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: a randomized controlled trial. J Adolesc Health 2014; 55: 6572.
69. Hillman, CH, Erickson, KI, Kramer, AF. Be smart, exercise your heart: exercise effects on brain and cognition. Nat Rev Neurosci 2008; 9: 5865.
70. Hillman, CH, Pontifex, MB, Castelli, DM, et al. Effects of the FITKids randomized controlled trial on executive control and brain function. Pediatrics 2014; 134: 10631071.



Altmetric attention score

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed