Skip to main content Accessibility help

Neuropsychological outcomes in CHD beyond childhood: a meta-analysis

  • Rónán Mills (a1), Christopher G. McCusker (a2), Chris Tennyson (a3) and Donncha Hanna (a4)



Risk for neurodevelopmental delay in infants and children with CHD is well established, but longer-term outcomes are equivocal. A meta-analysis was conducted to establish whether cognitive deficits remain beyond childhood – into teenage and young adult years.

Methods and results

A total of 18 unique samples, involving adolescents, teenagers, and adults with CHD significant enough to require invasive intervention, and sourced through searches of Web of Science, MEDLINE, CINAHL Plus, and PsychInfo, met the inclusion criteria. These included the use of standardised neuropsychology tests across 10 domains of cognitive functioning and the reporting of effect size differences with controls. Reports of patients with chromosomal or genetic abnormalities were excluded. Pooled effect sizes suggested no significant differences between CHD samples and controls in terms of general intellectual ability and verbal reasoning. However, small–medium effects sizes were noted (0.33–0.44) and were statistically significant within the domains of non-verbal reasoning, processing speed, attention, auditory–verbal memory, psychomotor abilities, numeracy, and literacy with executive functioning also emerging as significant when one study outlier was excluded. We also included quality assurance statistics including Cochran’s Q, T, and I2 statistics, leave-one-out analyses, and assessment of publication bias. These often suggested study variability, possibly related to the heterogeneity of diagnostic groups included, and different tests used to measure the same construct.


Heterogeneity indicated that moderators affect cognitive outcomes in CHD. Nevertheless, deficits across cognitive domains were discerned, which are likely to have functional impact and which should inform practice with this clinical population.


Corresponding author

Correspondence to: Dr C. McCusker, School of Applied Psychology, North Mall Enterprise Centre, University College Cork, Cork, Ireland. Tel: +353 21 490 4602; E-mail:


Hide All
1. Hoffman, JI, Kaplan, S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39: 18901900.
2. McCusker, CG, Casey, FC. (eds). Congenital Heart Disease and Neurodevelopment: Understanding and Improving Outcomes. Elsevier: Academic Press, London, 2016.
3. Snookes, SH, Gunn, JK, Eldridge, BJ, et al. A systematic review of motor and cognitive outcomes after early surgery for congenital heart disease. Pediatrics 2010; 125: e81827.
4. Hövels-Gürich, HH, McCusker, CG. Neurodevelopmental patterns in congenital heart disease across childhood – longitudinal studies from Europe. In: McCusker CG, Casey FC, (eds). Congenital Heart Disease and Neurodevelopment: Understanding and Improving Outcomes. Elsevier: Academic Press, London, 2016: pp. 41–53.
5. McCusker, CG, Armstrong, MP, Mullen, M, Doherty, NN, Casey, F. A sibling-controlled prospective study of outcomes at home and school in children with severe congenital heart disease. Cardiol Young 2013; 23: 507516.
6. 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.
7. Bellinger, DC, Rivkin, MJ, DeMaso, D, et al. Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging. Cardiol Young 2015; 25: 338347.
8. McCusker, CG, Doherty, NN, Molloy, B, et al. Determinants of neuropsychological and behavioural outcomes in early childhood survivors of congenital heart disease. Arch Dis Child 2007; 92: 137141.
9. Volpe, JJ. Encephalopathy of congenital heart disease – destructive and developmental effects intertwined. J Pediatr 2014; 164: 962965.
10. 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.
11. 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; 21: 116.
12. Tyagi, M, Austin, K, Stygall, J, Deanfield, J, Cullen, S, Newman, SP. What do we know about cognitive functioning in adult congenital heart disease? Cardiol Young 2014; 24: 1319.
13. Anderson, V, Spencer-Smith, M, Wood, A. Do children really recover better? Neurobehavioural plasticity after early brain insult. Brain 2011; 134: awr103.
14. Karsdorp, PA, Everaerd, W, Kindt, M, Mulder, BJ. Psychological and cognitive functioning in children and adolescents with congenital heart disease: a meta-analysis. J Pediatr Psychol 2007; 32: 527541.
15. Alden, B, Gilljam, T, Gillberg, C. Long‐term psychological outcome of children after surgery for transposition of the great arteries. Acta Paediatr 1998; 87: 405410.
16. Brewster, RC, King, TZ, Burns, TG, Drossner, DM, Mahle, WT. White matter integrity dissociates verbal memory and auditory attention span in emerging adults with congenital heart disease. J Int Neuropsychol Soc 2015; 21: 2233.
17. Heinrichs, AKM, Holschen, A, Krings, T, et al. Neurologic and psycho-intellectual outcome related to structural brain imaging in adolescents and young adults after neonatal arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 2014; 148: 21902199.
18. Kirshbom, PM, Flynn, TB, Clancy, RR, et al. Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 2005; 129: 10911097.
19. Matos, SM, Sarmento, S, Moreira, S, et al. Impact of fetal development on neurocognitive performance of adolescents with cyanotic and acyanotic congenital heart disease. Congnit Heart Dis 2014; 9: 373381.
20. Pereira, MMM, Areias, ME, Areias, JC, da Silva, ED, Peixoto, B. Neurocognitive implications of congenital heart diseases in adolescents. Acta Neuropsychologica 2011; 9: 351.
21. Quartermain, MD, Ittenbach, RF, Flynn, TB, et al. Neuropsychological status in children after repair of acyanotic congenital heart disease. Pediatrics 2010; 126: e3519.
22. Salzer-Muhar, U, Herle, M, Floquet, P, et al. Self-concept in male and female adolescents with congenital heart disease. Clin Pediatr (Phila) 2002; 41: 1724.
23. Schaefer, C, 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.
24. Spijkerboer, A, Utens, E, Bogers, A, Verhulst, F, Helbing, W. Long‐term intellectual functioning and school‐related behavioural outcomes in children and adolescents after invasive treatment for congenital heart disease. Br J Dev Psychol 2008; 26: 457470.
25. Utens, E, Bieman, H, Verhulst, F, Meijboom, F, Erdman, R, Hess, J. Psychopathology in young adults with congenital heart disease. Eur Heart J 1998; 19: 647651.
26. van der Rijken, R, Hulstijn-Dirkmaat, G, Kraaimaat, F, et al. Open-heart surgery at school age does not affect neurocognitive functioning. Eur Heart J 2008; 29: 26812688.
27. van der Rijken, R, Hulstijn‐Dirkmatt, G, Kraaimaat, F, Naburrs‐Kohrman, L, Daniels, O, Maassen, B. Evidence of impaired neurocognitive functioning in school‐age children awaiting cardiac surgery. Dev Med Child Neurol 2010; 52: 552558.
28. Visconti, KJ, Bichell, DP, Jonas, RA, Newburger, JW, Bellinger, DC. Developmental outcome after surgical versus interventional closure of secundum atrial septal defect in children. Circulation 1999; 100 (19 Suppl): II14550.
29. Wernovsky, G, Stiles, KM, Gauvreau, K, et al. Cognitive development after the fontan operation. Circulation 2000; 102: 883889.
30. Yang, L, Liu, M, Townes, BD. Neuropsychological and behavioral status of Chinese children with acyanotic congenital heart disease. Int J Neurosci 1994; 74: 109115.
31. Wechsler, D. Wechsler Intelligence Scale for Children–III. The Psychological Corporation, New York, 1991.
32. Wechsler, D. Wechsler Adult Intelligence Scale-III. The Psychological Corporation, New York, 1997.
33. Manly, T, Robertson, IH, Anderson, V, Nimmo-Smith, I. Test of Everyday Attention for Children. The Psychological Corporation, New York, 1998.
34. Cohen, M. Children’s Memory Scale. The Psychological Corporation, New York, 1997.
35. Delis, DC, Kaplan, E, Kramer, JH. Delis-Kaplan Executive Function System. The Psychological Corporation, New York, 2001.
36. Meyers, JE, Meyers, KR. Rey Complex Figure Test and Recognition Trial (RCFT). Psychological Assessment Resources, Odessa, FL, 1995.
37. Wilson, BA, Emslie, H, Evans, JJ, Alderman, N, Burgess, PW. Behavioural Assessment of the Dysexecutive Syndrome. Pearson Assessment, London, 1996.
38. Delis, D, Kaplan, E, Kramer, J, Ober, B. California Verbal Learning Test-II. The Psychological Corporation, San Antonio, TX, 2000.
39. Beery, KE. Developmental Test of Visual-Motor Integration: Administration and Scoring Manual. Follett Publishing, Chicago, IL, 1967.
40. The Psychological Corporation. Wechsler Individual Achievement Test. The Psychological Corporation, San Antonio, TX, 1992.
41. Cohen, J. Statistical Power Analysis for the Behavioral Sciences. Academic Press, New York, 1969.
42. Biostat Inc. Comprehensive meta-analysis, 2014 Englewood, NJ.
43. Borenstein, M. Introduction to Meta-Analysis. Wiley, Chichester, 2009.
44. Von Elm, E, Altman, DG, Egger, M, Pocock, SJ, Gotzsche, PC, Vandenbroucke, JP. The strengthening the reporting of observational studies in epidemiology (STROBE) statement: guidelines for reporting observational studies. Int J Surg 2014; 12:14951499.
45. Daliento, L, Mapelli, D, Russo, G, et al. Health related quality of life in adults with repaired tetralogy of Fallot: psychosocial and cognitive outcomes. Heart 2005; 91: 213218.
46. Opic, P, Roos-Hesselink, J, Cuypers, J, et al. Psychosocial functioning of adults with congenital heart disease: outcomes of a 30-43 year longitudinal follow-up. Clin Res Cardiol 2015; 104:388400.
47. van der Rijken, R, Hulstijn, W, Hulstijn-Dirkmaat, G, Daniëls, O, Maassen, B. Psychomotor slowness in school-age children with congenital heart disease. Dev Neuropsychol 2011; 36: 388402.
48. Moher, D, Liberati, A, Tetzlaff, J, Altman, DG, The PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. PLoS Med 2009; 6: e1000097.



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