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What do we know about cognitive functioning in adult congenital heart disease?

  • Manavi Tyagi (a1) (a2), Katie Austin (a3), Jan Stygall (a1), John Deanfield (a4), Shay Cullen (a4) and Stanton P. Newman (a1) (a5)...


With the advent of improved medical and surgical care in congenital heart disease, there has been an increase in the number of patients who survive into adulthood, giving rise to a new patient population ‘Adults with congenital heart disease’. In the past, morbidity and mortality were the primary concerns for this group. However, with improvements in outcome attention has shifted to other factors such as psychosocial and cognitive functioning. This paper reviews the literature on the cognitive functioning in adult congenital heart disease patients. A total of five relevant articles were retrieved via electronic searches of six databases, including MEDLINE, EMBASE, CINAHL, AMED, PsychINFO, and PubMed. The results displayed a consensus on the presence of some cognitive difficulties in adult congenital heart disease patients. The aetiology of cognitive dysfunctions appears to be multifactorial. The literature is limited by the very small number of studies looking at adults with congenital heart disease, with the majority focusing on cognitive functioning among children with congenital heart disease. However, the presence of cognitive dysfunctions and the resulting impact on the patient's day to day lives warrant for a more detailed and prospective research to enhance the understanding of its aetiology and impact.


Corresponding author

Correspondence to: Prof. S. P. Newman, Centre for Health Services Research, School of Health Sciences, City University London, Northampton Square, London EC1 V 0HB, United Kingdom. Tel: +44 207 040 5767; Fax: +44 207 040 0875; E-mail:


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Manavi Tyagi and Katie Austin are both first authors on this review with a shared first co-authorship.



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1. Warnes, C. The adult with congenital heart disease born to be bad? J Am Coll Cardiol 2005: 18.
2. Kenny, D, Stuart, AG. Long-term outcome of the child with congenital heart disease. Paediatr Child Health 2009: 3742.
3. Bedard, E, Shore, DF, Gatzoulis, MA. Adult congenital heart disease: a 2008 overview. Br Med Bull 2008: 151180.
4. Daliento, L, Mapelli, D, Volpe, B, et al. Measurement of cognitive outcome and quality of life in congenital heart disease. Heart 2006: 569574.
5. Bellinger, DC, Newburger, JW. Neuropsychological, psychosocial, and quality-of-life outcomes in children and adolescents with congenital heart disease. Prog Pediatr Cardiol 2010: 8792.
6. Massaro, AN, El-dib, M, Aly, H, et al. Factors associated with adverse neurodevelopmental outcomes in infants with congenital heart disease. Brain Dev 2008: 437446.
7. Miatton, M, De Wolf, D, Francois, K, Thiery, E, Vingerhoets, G. Neurocognitive consequences of surgically corrected congenital heart defects: a review. Neuropsychol Rev 2006: 6585.
8. Mahle, WT. Neurologic and cognitive outcomes in children with congenital heart disease. Curr Opin Pediatr 2001: 482486.
9. Karsdorp, PA, Everaerd, W, Kindt, M, et al. Psychological and cognitive functioning in children and adolescents with congenital heart disease: a meta-analysis. J Pediatr Psychol 2007: 527541.
10. Horner, T, Liberthson, R, Jellinek, MS. Psychosocial profile of adults with complex congenital heart disease. Mayo Clin Proc 2000: 3136.
11. Mcgrath, E, Wypij, D, Rappaport, L, et al. Prediction of IQ and achievement at age 8 years from neurodevelopmental status at age 1 year in children with D-transposition of the great arteries. Pediatrics 2004: e572e576.
12. Vingerhoets, G, De Soete, G, Jannes, C. Subjective complaints versus neuropsychological test performance after cardiopulmonary bypass. J Psychosom Res 1995: 843853.
13. Utens, EM, Verhulst, FC, Erdman, RA, et al. Psychosocial functioning of young adults after surgical correction for congenital heart disease in childhood: a follow-up study. J Psychosom Res 1994: 745758.
14. Utens, EM, Versluis, D, Verhulst, FC, et al. Psychopathology in young adults with congenital heart disease. Follow-up results. Eur Heart J 1998: 647651.
15. 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: 213218.
16. Wernovsky, G, Stiles, K, Gauvreau, K, et al. Cognitive development after the Fontan operation. Circulation 2000: 883889.
17. Eide, MG, Skjaerven, R, Irgens, LM. Associations of birth defects with adult intellectual performance, disability and mortality: population-based cohort study. Pediatr Res 2006: 848853.
18. Grech, V, Gatt, M. Syndromes and malformations associated with congenital heart disease in a population-based study. Int J Cardiol 1999: 151156.
19. Meberg, A, Hals, J, Thaulow, E. Congenital heart defects – chromosomal anomalies, syndromes and extracardiac malformations. Acta Paediatrica 2007: 11421145.
20. Bellinger, D, Wypij, D, Duplessis, A, 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: 13851396.
21. Wypij, D, Newburger, JW, Rappaport, LA, et al. The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: The Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003: 13971403.
22. Newburger, JW, Silbert, AR, Buckley, LP, et al. Cognitive function and age at repair of transposition of the great arteries in children. N Engl J Med 1984: 14951499.
23. Newburger, JW, Wypij, D, Bellinger, DC, et al. Length of stay after infant heart surgery is related to cognitive outcome at age 8 years. J Pediatr 2003: 6773.
24. Sahu, B, Chauhan, S, Kiran, U, et al. Neuropsychological function in children with cyanotic heart disease undergoing corrective cardiac surgery: effect of two different rewarming strategies. Eur J Cardiothorac Surg 2009: 505510.


What do we know about cognitive functioning in adult congenital heart disease?

  • Manavi Tyagi (a1) (a2), Katie Austin (a3), Jan Stygall (a1), John Deanfield (a4), Shay Cullen (a4) and Stanton P. Newman (a1) (a5)...


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