Neuropsychological Outcomes and Posttraumatic Stress Disorder in Adults with Congenital Heart Disease

  • Adrienne H. KovacsEmail author
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)


There exists an association between physical and psychosocial factors, which demands that we attend to both in order to optimize patient outcomes. Compared to healthy peers, as a group, adults with congenital heart disease are at elevated risk of neurocognitive deficits, mood and anxiety disorders, and posttraumatic stress disorder. These psychosocial difficulties have historically been under recognized and undertreated in the outpatient setting, likely even more so in the inpatient setting. This chapter provides an overview of the psychosocial challenges faced by many hospitalized adults with congenital heart disease, with a particular emphasis on neuropsychological outcomes and trauma experiences. The objective is to improve providers’ confidence in their ability to more effectively recognize and manage these challenges in the inpatient setting.


Adult congenital heart disease Intensive care Critical care Neurocognitive Executive functions Psychosocial Depression Anxiety Posttraumatic stress disorder Mental health 


  1. 1.
    Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor 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(9):1143–72.PubMedCrossRefGoogle Scholar
  2. 2.
    Wilson WM, Smith-Parrish M, Marino BS, Kovacs AH. Neurodevelopmental and psychosocial outcomes across the congenital heart disease lifespan. Prog Pediatr Cardiol. 2015;39:113–8.CrossRefGoogle Scholar
  3. 3.
    Utens EM, Verhulst FC, Erdman RA, Meijboom FJ, Duivenvoorden HJ, Bos E, et al. Psychosocial functioning of young adults after surgical correction for congenital heart disease in childhood: a follow-up study. J Psychosom Res. 1994;38(7):745–58.PubMedCrossRefGoogle Scholar
  4. 4.
    Wernovsky G, Stiles KM, Gauvreau K, Gentles TL, du Plessis AJ, Bellinger DC, et al. Cognitive development after the Fontan operation. Circulation. 2000;102(8):883–9.PubMedCrossRefGoogle Scholar
  5. 5.
    Daliento L, Mapelli D, Russo G, Scarso P, Limongi F, Iannizzi P, et al. Health related quality of life in adults with repaired tetralogy of Fallot: psychosocial and cognitive outcomes. Heart. 2005;91(2):213–8.PubMedPubMedCentralCrossRefGoogle Scholar
  6. 6.
    Ilardi D, Ono KE, McCartney R, Book W, Stringer AY. Neurocognitive functioning in adults with congenital heart disease. Congenit Heart Dis. 2016;12(2):166–73.PubMedCrossRefGoogle Scholar
  7. 7.
    Tyagi M, Fteropoulli T, Hurt CS, Hirani SP, Rixon L, Davies A, et al. Cognitive dysfunction in adult CHD with different structural complexity. Cardiol Young. 2017;27(5):851–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Klouda L, Franklin WJ, Saraf A, Parekh DR, Schwartz DD. Neurocognitive and executive functioning in adult survivors of congenital heart disease. Congenit Heart Dis. 2017;12(1):91–8.PubMedCrossRefGoogle Scholar
  9. 9.
    Murphy LK, Compas BE, Reeslund KL, Gindville MC, Mah ML, Markham LW, et al. Cognitive and attentional functioning in adolescents and young adults with Tetralogy of Fallot and d-transposition of the great arteries. Child Neuropsychol. 2017;23(1):99–110.PubMedCrossRefGoogle Scholar
  10. 10.
    Diamond A. Executive functions. Annu Rev Psychol. 2013;64:135–68.PubMedCrossRefGoogle Scholar
  11. 11.
    Calderon J, Bellinger DC. Executive function deficits in congenital heart disease: why is intervention important? Cardiol Young. 2015;25(7):1238–46.PubMedCrossRefGoogle Scholar
  12. 12.
    Marino BS, Beebe D, Cassedy A, Riedel M, Burger M, Medek S, et al. Executive functioning, gross motor ability and mood are key drivers of poorer quality of life in child and adolescent survivors with complex congenital heart disease. J Am Coll Cardiol. 2011;57:E421.CrossRefGoogle Scholar
  13. 13.
    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. 2015;21(1):34–49.PubMedCrossRefGoogle Scholar
  14. 14.
    Sable C, Foster E, Uzark K, Bjornsen K, Canobbio MM, Connolly HM, et al. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation. 2011;123(13):1454–85.PubMedCrossRefGoogle Scholar
  15. 15.
    Kovacs AH, McCrindle BW. So hard to say goodbye: transition from paediatric to adult cardiology care. Nat Rev Cardiol. 2014;11(1):51–62.PubMedCrossRefGoogle Scholar
  16. 16.
    Kovacs AH, Webb GD. Preparing pediatric patients for adult care: are we ready? J Pediatr. 2015;167(6):1194–5.PubMedCrossRefGoogle Scholar
  17. 17.
    Duke DC, Harris MA. Executive function, adherence, and glycemic control in adolescents with type 1 diabetes: a literature review. Curr Diab Rep. 2014;14(10):532.PubMedCrossRefGoogle Scholar
  18. 18.
    Bellinger DC, Wypij D, Rivkin MJ, DeMaso DR, Robertson RL Jr, Dunbar-Masterson C, 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(12):1361–9.PubMedPubMedCentralCrossRefGoogle Scholar
  19. 19.
    Bellinger DC, Rivkin MJ, DeMaso D, Robertson RL, Stopp C, Dunbar-Masterson C, et al. Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging. Cardiol Young. 2015;25(2):338–47.PubMedCrossRefGoogle Scholar
  20. 20.
    Geyer S, Norozi K, Buchhorn R, Wessel A. Chances of employment in women and men after surgery of congenital heart disease: comparisons between patients and the general population. Congenit Heart Dis. 2009;4(1):25–33.PubMedCrossRefGoogle Scholar
  21. 21.
    Karsenty C, Maury P, Blot-Souletie N, Ladouceur M, Leobon B, Senac V, et al. The medical history of adults with complex congenital heart disease affects their social development and professional activity. Arch Cardiovasc Dis. 2015;108(11):589–97.PubMedCrossRefGoogle Scholar
  22. 22.
    Sluman MA, Apers S, Bouma BJ, van Melle JP, Peels CH, Post MC, et al. Uncertainties in insurances for adults with congenital heart disease. Int J Cardiol. 2015;186:93–5.PubMedCrossRefGoogle Scholar
  23. 23.
    Bellinger DC. Are children with congenital cardiac malformations at increased risk of deficits in social cognition? Cardiol Young. 2008;18(1):3–9.PubMedCrossRefGoogle Scholar
  24. 24.
    Bellinger DC. Theory of mind deficits in children with congenital heart disease. Dev Med Child Neurol. 2010;52(12):1079–80.PubMedCrossRefGoogle Scholar
  25. 25.
    Kovacs AH, Saidi AS, Kuhl EA, Sears SF, Silversides C, Harrison JL, et al. Depression and anxiety in adult congenital heart disease: predictors and prevalence. Int J Cardiol. 2009;137(2):158–64.PubMedCrossRefGoogle Scholar
  26. 26.
    Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Enomoto J, et al. Quality of life of adults with congenital heart disease in 15 countries: evaluating country-specific characteristics. J Am Coll Cardiol. 2016;67(19):2237–45.PubMedCrossRefGoogle Scholar
  27. 27.
    Moons P, Van Deyk K, Marquet K, Raes E, De Bleser L, Budts W, et al. Individual quality of life in adults with congenital heart disease: a paradigm shift. Eur Heart J. 2005;26(3):298–307.PubMedCrossRefGoogle Scholar
  28. 28.
    Marelli A, Miller SP, Marino BS, Jefferson AL, Newburger JW. Brain in congenital heart disease across the lifespan: the cumulative burden of injury. Circulation. 2016;133(20):1951–62.PubMedPubMedCentralCrossRefGoogle Scholar
  29. 29.
    Lanz J, Brophy JM, Therrien J, Kaouache M, Guo L, Marelli AJ. Stroke in adults with congenital heart disease: incidence, cumulative risk, and predictors. Circulation. 2015;132(25):2385–94.PubMedCrossRefGoogle Scholar
  30. 30.
    Eggermont LH, de Boer K, Muller M, Jaschke AC, Kamp O, Scherder EJ. Cardiac disease and cognitive impairment: a systematic review. Heart. 2012;98(18):1334–40.PubMedCrossRefGoogle Scholar
  31. 31.
    Vogels RL, Oosterman JM, van Harten B, Scheltens P, van der Flier WM, Schroeder-Tanka JM, et al. Profile of cognitive impairment in chronic heart failure. J Am Geriatr Soc. 2007;55(11):1764–70.PubMedCrossRefGoogle Scholar
  32. 32.
    Vogels RL, Scheltens P, Schroeder-Tanka JM, Weinstein HC. Cognitive impairment in heart failure: a systematic review of the literature. Eur J Heart Fail. 2007;9(5):440–9.PubMedCrossRefGoogle Scholar
  33. 33.
    Gyurak A, Goodkind MS, Kramer JH, Miller BL, Levenson RW. Executive functions and the down-regulation and up-regulation of emotion. Cogn Emot. 2012;26(1):103–18.PubMedCrossRefGoogle Scholar
  34. 34.
    Cacciatore F, Abete P, Ferrara N, Calabrese C, Napoli C, Maggi S, et al. Congestive heart failure and cognitive impairment in an older population. Osservatorio Geriatrico Campano Study Group. J Am Geriatr Soc. 1998;46(11):1343–8.PubMedCrossRefGoogle Scholar
  35. 35.
    Trojano L, Antonelli Incalzi R, Acanfora D, Picone C, Mecocci P, Rengo F, et al. Cognitive impairment: a key feature of congestive heart failure in the elderly. J Neurol. 2003;250(12):1456–63.PubMedCrossRefGoogle Scholar
  36. 36.
    Pressler SJ. Cognitive functioning and chronic heart failure: a review of the literature (2002–July 2007). J Cardiovasc Nurs. 2008;23(3):239–49.PubMedCrossRefGoogle Scholar
  37. 37.
    Zuccala G, Onder G, Pedone C, Carosella L, Pahor M, Bernabei R, et al. Hypotension and cognitive impairment: selective association in patients with heart failure. Neurology. 2001;57(11):1986–92.PubMedCrossRefGoogle Scholar
  38. 38.
    Kalantarian S, Ruskin JN. Cognitive impairment associated with atrial fibrillation—in response. Ann Intern Med. 2013;158(11):849.PubMedCrossRefGoogle Scholar
  39. 39.
    Newman MF, Kirchner JL, Phillips-Bute B, Gaver V, Grocott H, Jones RH, et al. Longitudinal assessment of neurocognitive function after coronary-artery bypass surgery. N Engl J Med. 2001;344(6):395–402.PubMedCrossRefGoogle Scholar
  40. 40.
    Pandharipande PP, Girard TD, Jackson JC, Morandi A, Thompson JL, Pun BT, et al. Long-term cognitive impairment after critical illness. N Engl J Med. 2013;369(14):1306–16.PubMedPubMedCentralCrossRefGoogle Scholar
  41. 41.
    Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol. 1996;28(3):768–72.PubMedCrossRefGoogle Scholar
  42. 42.
    Jackson JL, Misiti B, Bridge JA, Daniels CJ, Vannatta K. Emotional functioning of adolescents and adults with congenital heart disease: a meta-analysis. Congenit Heart Dis. 2015;10(1):2–12.PubMedCrossRefGoogle Scholar
  43. 43.
    Horner T, Liberthson R, Jellinek MS. Psychosocial profile of adults with complex congenital heart disease. Mayo Clin Proc. 2000;75(1):31–6.PubMedCrossRefGoogle Scholar
  44. 44.
    Bromberg JI, Beasley PJ, D'Angelo EJ, Landzberg M, DeMaso DR. Depression and anxiety in adults with congenital heart disease: a pilot study. Heart Lung. 2003;32(2):105–10.PubMedCrossRefGoogle Scholar
  45. 45.
    White KS, Pardue C, Ludbrook P, Sodhi S, Esmaeeli A, Cedars A. Cardiac denial and psychological predictors of cardiac care adherence in adults with congenital heart disease. Behav Modif. 2016;40(1–2):29–50.PubMedCrossRefGoogle Scholar
  46. 46.
    Kourkoveli P, Rammos S, Parissis J, Maillis A, Kremastinos D, Paraskevaidis I. Depressive symptoms in patients with congenital heart disease: incidence and prognostic value of self-rating depression scales. Congenit Heart Dis. 2015;10(3):240–7.PubMedCrossRefGoogle Scholar
  47. 47.
    Kim YY, Gauvreau K, Bacha EA, Landzberg MJ, Benavidez OJ. Resource use among adult congenital heart surgery admissions in pediatric hospitals: risk factors for high resource utilization and association with inpatient death. Circ Cardiovasc Qual Outcomes. 2011;4(6):634–9.PubMedCrossRefGoogle Scholar
  48. 48.
    Lichtman JH, Bigger JT Jr, Blumenthal JA, Frasure-Smith N, Kaufmann PG, Lesperance F, et al. Depression and coronary heart disease: recommendations for screening, referral, and treatment: a science advisory from the American Heart Association Prevention Committee of the Council on Cardiovascular Nursing, Council on Clinical Cardiology, Council on Epidemiology and Prevention, and Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Psychiatric Association. Circulation. 2008;118(17):1768–75.PubMedCrossRefGoogle Scholar
  49. 49.
    Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults with Congenital Heart Disease). Developed in Collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;52(23):e1–121.CrossRefGoogle Scholar
  50. 50.
    Thombs BD, Roseman M, Coyne JC, de Jonge P, Delisle VC, Arthurs E, et al. Does evidence support the American Heart Association's recommendation to screen patients for depression in cardiovascular care? An updated systematic review. PLoS One. 2013;8(1):e52654.PubMedPubMedCentralCrossRefGoogle Scholar
  51. 51.
    Ferguson M, Kovacs AH. An integrated adult congenital heart disease psychology service. Congenit Heart Dis. 2016;11(5):444–51.PubMedCrossRefGoogle Scholar
  52. 52.
    Kovacs AH, Bendell KL, Colman J, Harrison JL, Oechslin E, Silversides C. Adults with congenital heart disease: psychological needs and treatment preferences. Congenit Heart Dis. 2009;4(3):139–46.PubMedCrossRefGoogle Scholar
  53. 53.
    Page MG, Kovacs AH, Irvine J. How do psychosocial challenges associated with living with congenital heart disease translate into treatment interests and preferences? A qualitative approach. Psychol Health. 2012;27(11):1260–70.PubMedCrossRefGoogle Scholar
  54. 54.
    Butler AC, Chapman JE, Forman EM, Beck AT. The empirical status of cognitive-behavioral therapy: a review of meta-analyses. Clin Psychol Rev. 2006;26(1):17–31.PubMedCrossRefGoogle Scholar
  55. 55.
    Hofmann SG, Smits JAJ. Cognitive-behavioral therapy for adult anxiety disorders: a meta-analysis of randomized placebo-controlled trials. J Clin Psychiatry. 2008;69(4):621–32.PubMedPubMedCentralCrossRefGoogle Scholar
  56. 56.
    Kovacs AH, Bandyopadhyay M, Grace SL, Kentner AC, Nolan RP, Silversides CK, et al. Adult Congenital Heart Disease-Coping And REsilience (ACHD-CARE): rationale and methodology of a pilot randomized controlled trial. Contemp Clin Trials. 2015;45(Pt B):385–93.PubMedCrossRefGoogle Scholar
  57. 57.
    Morton L. Can interpersonal psychotherapy (IPT) meet the psychosocial cost of life gifted by medical intervention. Couns Psychol Rev. 2011;26(3):75–86.Google Scholar
  58. 58.
    Abbott RA, Whear R, Rodgers LR, Bethel A, Coon JT, Kuyken W, et al. Effectiveness of mindfulness-based stress reduction and mindfulness based cognitive therapy in vascular disease: a systematic review and meta-analysis of randomised controlled trials. J Psychosom Res. 2014;76(5):341–51.PubMedCrossRefGoogle Scholar
  59. 59.
    Bohlmeijer E, Prenger R, Taal E, Cuijpers P. The effects of mindfulness-based stress reduction therapy on mental health of adults with a chronic medical disease: a meta-analysis. J Psychosom Res. 2010;68(6):539–44.PubMedCrossRefGoogle Scholar
  60. 60.
    Kabat-Zinn J. Full catastrophe living: using the wisdom of your body and mind to face stress, pain, and illness (15th anniversary ed.). New York: Delta Trade Paperback/Bantam Dell; 2005. 471-xxxiii p.Google Scholar
  61. 61.
    Hare DL, Toukhsati SR, Johansson P, Jaarsma T. Depression and cardiovascular disease: a clinical review. Eur Heart J. 2014;35(21):1365–72.PubMedCrossRefGoogle Scholar
  62. 62.
    Nezafati MH, Vojdanparast M, Nezafati P. Antidepressants and cardiovascular adverse events: a narrative review. ARYA Atheroscler. 2015;11(5):295–304.PubMedPubMedCentralGoogle Scholar
  63. 63.
    Dornelas EA. Behavioral cardiology: toward the development of integrated treatment models. In: Dornelas EA, editor. Stress proof the heart: behavioral interventions for cardiac patients. New York: Springer; 2012. p. 389–400.CrossRefGoogle Scholar
  64. 64.
    Soufi A, Colman JM, Li Q, Oechslin EN, Kovacs AH. Review of non-elective hospitalisations of adults with CHD. Cardiol Young. 2017;27(9):1764–70.PubMedCrossRefGoogle Scholar
  65. 65.
    American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 4th ed., text rev ed. Washington: Author; 2000.Google Scholar
  66. 66.
    American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 5th ed. Washington, DC: Author; 2013.CrossRefGoogle Scholar
  67. 67.
    Shalev A, Liberzon I, Marmar C. Post-traumatic stress disorder. N Engl J Med. 2017;376(25):2459–69.PubMedCrossRefGoogle Scholar
  68. 68.
    Kessler RC, Chiu WT, Demler O, Merikangas KR, Walters EE. Prevalence, severity, and comorbidity of 12-month DSM-IV disorders in the National Comorbidity Survey Replication. Arch Gen Psychiatry. 2005;62(6):617–27.PubMedPubMedCentralCrossRefGoogle Scholar
  69. 69.
    Keane TM, Marshall AD, Taft CT. Posttraumatic stress disorder: etiology, epidemiology, and treatment outcome. Annu Rev Clin Psychol. 2006;2:161–97.PubMedCrossRefGoogle Scholar
  70. 70.
    Kessler RC, Berglund P, Demler O, Jin R, Merikangas KR, Walters EE. Lifetime prevalence and age-of-onset distributions of DSM-IV disorders in the National Comorbidity Survey Replication. Arch Gen Psychiatry. 2005;62(6):593–602.PubMedPubMedCentralCrossRefGoogle Scholar
  71. 71.
    Vilchinsky N, Ginzburg K, Fait K, Foa EB. Cardiac-disease-induced PTSD (CDI-PTSD): a systematic review. Clin Psychol Rev. 2017;55:92–106.PubMedCrossRefGoogle Scholar
  72. 72.
    Ladwig KH, Baumert J, Marten-Mittag B, Kolb C, Zrenner B, Schmitt C. Posttraumatic stress symptoms and predicted mortality in patients with implantable cardioverter-defibrillators: results from the prospective living with an implanted cardioverter-defibrillator study. Arch Gen Psychiatry. 2008;65(11):1324–30.PubMedCrossRefGoogle Scholar
  73. 73.
    Edmondson D, Richardson S, Falzon L, Davidson KW, Mills MA, Neria Y. Posttraumatic stress disorder prevalence and risk of recurrence in acute coronary syndrome patients: a meta-analytic review. PLoS One. 2012;7(6):e38915.PubMedPubMedCentralCrossRefGoogle Scholar
  74. 74.
    Dao TK, Chu D, Springer J, Gopaldas RR, Menefee DS, Anderson T, et al. Clinical depression, posttraumatic stress disorder, and comorbid depression and posttraumatic stress disorder as risk factors for in-hospital mortality after coronary artery bypass grafting surgery. J Thorac Cardiovasc Surg. 2010;140(3):606–10.PubMedCrossRefGoogle Scholar
  75. 75.
    Deng LX, Khan AM, Drajpuch D, Fuller S, Ludmir J, Mascio CE, et al. Prevalence and correlates of post-traumatic stress disorder in adults with congenital heart disease. Am J Cardiol. 2016;117(5):853–7.PubMedCrossRefGoogle Scholar
  76. 76.
    Eslami B. Correlates of posttraumatic stress disorder in adults with congenital heart disease. Congenit Heart Dis. 2017;12(3):357–63.PubMedCrossRefGoogle Scholar
  77. 77.
    Vilchinsky N. Unrecognized, undiagnosed, and untreated: cardiac-disease-induced PTSD among patients’ partners. Front Psychol. 2017;8:1265.PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Knight Cardiovascular Institute, Oregon Health and Science UniversityPortlandUSA

Personalised recommendations