Journal of Genetic Counseling

, Volume 21, Issue 1, pp 72–84 | Cite as

Predictors of Heart-Focused Anxiety in Patients Undergoing Genetic Investigation and Counseling of Long QT Syndrome or Hypertrophic Cardiomyopathy: A One Year Follow-up

  • Anniken Hamang
  • Geir Egil Eide
  • Berit Rokne
  • Karin Nordin
  • Cathrine Bjorvatn
  • Nina Øyen
Original Research

Abstract

Since Long QT syndrome and Hypertrophic cardiomyopathy are inherited cardiac disorders that may cause syncope, palpitations, serious arrhythmias, and sudden cardiac death, at-risk individuals may experience heart-focused anxiety. In a prospective multi-site study, 126 Norwegian patients attending genetic counseling were followed 1 year with multiple administration of questionnaires, including the Cardiac Anxiety Questionnaire, measuring three distinct symptoms of heart-focused anxiety- avoidance, attention, and fear—in mixed linear analyses. Overall, at 1-year follow-up, patients with clinical diagnosis as compared to patients at genetic risk had significantly higher scores of avoidance (p < .002), attention (p < .005), and fear (p < .007). Sudden cardiac death in close relatives, uncertainty whether other relatives previously had undergone genetic testing, patients’ perceived general health, self-efficacy expectations and procedural satisfaction with genetic counseling were influential in predicting the different symptoms of heart-focused anxiety over time.

Keywords

Long QT syndrome Hypertrophic cardiomyopathy Genetic counseling Heart-focused anxiety Sudden cardiac death 

Notes

Acknowledgements

The authors thank all patients who participated in the study. We also acknowledge all helpful assistance from the Genetic Departments in Oslo, Bergen and Trondheim. The project was supported financially by Western Norway Regional Health Authority and the University of Bergen.

References

  1. Act of 5 December 2003 No. 100 relating to the application of biotechnology in human medicine, etc. Cf. earlier Acts of 5 August 1994 No. 56 and 12 June 1987 No. 68 http://www.ub.uio.no/ujur/ulovdata/lov-20031205-100-eng.pdf
  2. Aicher, D., Holz, A., Feldner, S., Köllner, V., & Schäfers, H. (2011). Quality of life after aortic valve surgery: Replacement versus reconstruction. J Thorac Cardiovas Surg http://www.sciencedirect.com/science/article/pii/S0022522311001796, doi: 10.1016/j.jtcvs.2011.02.006
  3. Andersen, J., Øyen, N., Bjorvatn, C., & Gjengedal, E. (2008). Living with long QT syndrome: a qualitative study of coping with increased risk of sudden cardiac death. Journal of Genetic Counseling, 17(5), 489–498.PubMedCrossRefGoogle Scholar
  4. Bjorvatn, C., Eide, G. E., Hanestad, B. R., & Havik, O. E. (2008). Anxiety and depression among subjects attending genetic counseling for hereditary cancer. Patient Education and Counseling, 71(2), 234–243.PubMedCrossRefGoogle Scholar
  5. Bjorvatn, C., Eide, G. E., Hanestad, B. R., Hamang, A., & Havik, O. E. (2009). Intrusion and avoidance in subjects undergoing genetic investigation and counseling for hereditary cancer. Supportive Care in Cancer, 17(11), 1371–1381.PubMedCrossRefGoogle Scholar
  6. Bos, J. M., Towbin, J. A., & Ackerman, M. J. (2009). Diagnostic, prognostic, and therapeutic implications of genetic testing for hypertrophic cardiomyopathy. Journal of the American College of Cardiology, 54(3), 201–211.PubMedCrossRefGoogle Scholar
  7. Carroll, D. L., Hamilton, G. A., & McGovern, B. A. (1999). Changes in health status and quality of life and the impact of uncertainty in patients who survive life-threatening arrhythmias. Heart & Lung, 28(4), 251–260.CrossRefGoogle Scholar
  8. Christiaans, I., van Langen, I. M., Birnie, E., Bonsel, G. J., Wilde, A. A., & Smets, M. (2009). Quality of life and psychological distress in hypertrophic cardiomyopathy mutation carriers: a cross-sectional cohort study. American Journal of Medical Genetics, 149A(4), 602–612.PubMedCrossRefGoogle Scholar
  9. Crotti, L., Celano, G., Dagradi, F., & Schwartz, P. J. (2008). Congenital long QT syndrome. Orphanet J Rare Dis, 3,18. http://www.ojrd.com/content/3/1/18, doi: 10.1186/1750-1172-3-18
  10. Eifert, G. H. (1992). Cardiophobia: a paradigmatic behavioural model of heart-focused anxiety and non-anginal chest pain. Behaviour Research and Therapy, 30(4), 329–345.PubMedCrossRefGoogle Scholar
  11. Eifert, G. H., & Forsyth, J. P. (1996). Heart-focused and general illness fears in relation to parental medical history and separation experiences. Behaviour Research and Therapy, 34(9), 735–739.PubMedCrossRefGoogle Scholar
  12. Eifert, G. H., Thompson, R. N., Zvolensky, M. J., Edwards, K., Frazer, N. L., Haddad, J. W., et al. (2000a). The cardiac anxiety questionnaire: development and preliminary validity. Behaviour Research and Therapy, 38(10), 1039–1053.CrossRefGoogle Scholar
  13. Eifert, G. H., Zvolensky, M. J., & Lejuez, C. W. (2000b). Heart-focused anxiety and chest pain: a conceptual and clinical review. Clinical Psychology-Science and Practice, 7(4), 403–417.CrossRefGoogle Scholar
  14. Elliott, P., & Spirito, P. (2008). Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice. Heart, 94(10), 1269–1275.PubMedCrossRefGoogle Scholar
  15. Fayers, P. M., & Sprangers, M. A. (2002). Understanding self-rated health. Lancet, 359(9302), 187–188.PubMedCrossRefGoogle Scholar
  16. Fulda, K. G., & Lykens, K. (2006). Ethical issues in predictive genetic testing: a public health perspective. Journal of Medical Ethics, 32(3), 143–147.PubMedCrossRefGoogle Scholar
  17. Garratt, C. J., Elliott, P., Behr, E., Camm, A. J., Cowan, C., Cruickshank, S., et al. (2010). Heart Rhythm UK position statement on clinical indications for implantable cardioverter defibrillators in adult patients with familial sudden cardiac death syndromes. Europace, 12(8), 1156–1175.PubMedCrossRefGoogle Scholar
  18. Goldenberg, I., & Moss, A. J. (2008). Long QT Syndrome. Journal of the American College of Cardiology, 51(24), 2291–2300.PubMedCrossRefGoogle Scholar
  19. Gregor, K. L., Zvolensky, M. J., & Yartz, A. R. (2005). Perceived health among individuals with panic disorder: associations with affective vulnerability and psychiatric disability. The Journal of Nervous and Mental Disease, 193(10), 697–699.PubMedCrossRefGoogle Scholar
  20. Gueorguieva, R., & Krystal, J. H. (2004). Move over ANOVA: progress in analyzing repeated-measures data and its reflection in papers published in the Archives of General Psychiatry. Archives of General Psychiatry, 61(3), 310–317.PubMedCrossRefGoogle Scholar
  21. Hamang, A., Solberg, B., Bjorvatn, C., Greve, G., & Oyen, N. (2009). Genetic counseling in congenital long QT syndrome. Tidsskrift for den Norske Lægeforening, 129(12), 1226–1229.PubMedCrossRefGoogle Scholar
  22. Hamang, A., Eide, G. E., Nordin, K., Rokne, B., Bjorvatn, C., & Oyen, N. (2010). Health status in patients at risk of inherited arrhythmias and sudden unexpected death compared to the general population. BMC Med Gen, 11(1), 27. http://www.biomedcentral.com/1471-2350/11/27
  23. Hendriks, K. S., Grosfeld, F. J., van Tintelen, J. P., van Langen, I. M., Wilde, A. A., van den Bout, J., et al. (2005a). Can parents adjust to the idea that their child is at risk for a sudden death?: psychological impact of risk for long QT syndrome. American Journal of Medical Genetics, 138A(2), 107–112.CrossRefGoogle Scholar
  24. Hendriks, K. S., Grosfeld, F. J., Wilde, A. A., van den Bout, J., van Langen, I. M., van Tintelen, J. P., et al. (2005b). High distress in parents whose children undergo predictive testing for long QT syndrome. Community Genetics, 8(2), 103–113.CrossRefGoogle Scholar
  25. Hendriks, K. S., Hendriks, M. M., Birnie, E., Grosfeld, F. J., Wilde, A. A., van den Bout, J., et al. (2008). Familial disease with a risk of sudden death: a longitudinal study of the psychological consequences of predictive testing for long QT syndrome. Heart Rhythm, 5(5), 719–724.PubMedCrossRefGoogle Scholar
  26. Hoyer, J., Eifert, G. H., Einsle, F., Zimmermann, K., Krauss, S., Knaut, M., et al. (2008). Heart-focused anxiety before and after cardiac surgery. Journal of Psychosomatic Research, 64(3), 291–297.PubMedCrossRefGoogle Scholar
  27. Lampert, R., Salberg, L., & Burg, M. (2010). Emotional stress triggers symptoms in hypertrophic cardiomyopathy: a survey of the Hypertrophic cardiomyopathy association. PACE, 33, 1047–1053.PubMedCrossRefGoogle Scholar
  28. Loge, J. H., & Kaasa, S. (1998). Short form 36 (SF-36) health survey: normative data from the general Norwegian population. Scandinavian Journal of Social Medicine, 26(4), 250–258.PubMedGoogle Scholar
  29. Loge, J. H., Kaasa, S., Hjermstad, M. J., & Kvien, T. K. (1998). Translation and performance of the Norwegian SF-36 Health Survey in patients with rheumatoid arthritis. I. Data quality, scaling assumptions, reliability, and construct validity. Journal of Clinical Epidemiology, 51(11), 1069–1076.PubMedCrossRefGoogle Scholar
  30. Maron, B. J. (2002). Hypertrophic cardiomyopathy: a systematic review. JAMA, 287(10), 1308–1320.PubMedCrossRefGoogle Scholar
  31. Michels, M., Soliman, O. I., Phefferkorn, J., Hoedemaekers, Y. M., Kofflard, M. J., Dooijes, D., et al. (2009). Disease penetrance and risk stratification for sudden cardiac death in asymptomatic hypertrophic cardiomyopathy mutation carriers. European Heart Journal, 30, 2593–2598.PubMedCrossRefGoogle Scholar
  32. Norekval, T. M., Fridlund, B., Rokne, B., Segadal, L., Wentzel-Larsen, T., & Nordrehaug, J. E. (2010). Patient-reported outcomes as predictors of 10-year survival in women after acute myocardial infarction. Health Qual Life Out, 8, 140. http://www.hqlo.com/content/8/1/140
  33. Priori, S. G., Napolitano, C., & Schwartz, P. J. (1999). Low penetrance in the long-QT syndrome: clinical impact. Circulation, 99(4), 529–533.PubMedGoogle Scholar
  34. Priori, S. G., Schwartz, P. J., Napolitano, C., Bloise, R., Ronchetti, E., Grillo, M., et al. (2003). Risk stratification in the long-QT syndrome. The New England Journal of Medicine, 348(19), 1866–1874.PubMedCrossRefGoogle Scholar
  35. Schwartz, P. J., Priori, S. G., Spazzolini, C., Moss, A. J., Vincent, G. M., Napolitano, C., et al. (2001). Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation, 103(1), 89–95.PubMedGoogle Scholar
  36. Shiloh, S., Avdor, O., & Goodman, R. M. (1990). Satisfaction with genetic counseling: dimensions and measurement. American Journal of Medical Genetics, 37(4), 522–529.PubMedCrossRefGoogle Scholar
  37. Splawski, I., Shen, J., Timothy, K. W., Lehmann, M. H., Priori, S., Robinson, J. L., et al. (2000). Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation, 102(10), 1178–1185.PubMedGoogle Scholar
  38. Tercyak, K. P., DeMarco, T. A., Mars, B. D., & Peshkin, B. N. (2004). Women’s satisfaction with genetic counseling for hereditary breast-ovarian cancer: psychological aspects. American Journal of Medical Genetics, 131A, 36–41.CrossRefGoogle Scholar
  39. Vincent, G. M. (2005). The Long QT and Brugada syndromes: causes of unexpected syncope and sudden cardiac death in children and young adults. Seminars in Pediatric Neurology, 12(1), 15–24.PubMedCrossRefGoogle Scholar
  40. Ware, J. E., Jr., & Gandek, B. (1998). Overview of the SF-36 health survey and the international quality of life assessment (IQOLA) project. Journal of Clinical Epidemiology, 51(11), 903–912.PubMedCrossRefGoogle Scholar
  41. Ware, J. E., Kosinki, M., & Gandek, B. (2000). SF-36 Health Survey: manual & interpretation guide (2nd ed.). Lincoln, Rhode Island: QualityMetric Inc.Google Scholar
  42. Watkins, H., Ashrafian, H., & Redwood, C. (2011). Inherited cardiomyopathies. The New England Journal of Medicine, 364(17), 1643–1656.PubMedCrossRefGoogle Scholar
  43. Yartz, A. R., Zvolensky, M. J., Gregor, K., Feldner, M. T., & Leen-Feldner, E. W. (2005). Health perception is a unique predictor of anxiety symptoms in non-clinical participants. Cognitive Behaviour Therapy, 34(2), 65–74.PubMedCrossRefGoogle Scholar
  44. Zareba, W., Moss, A. J., Schwartz, P. J., Vincent, G. M., Robinson, J. L., Priori, S. G., et al. (1998). Influence of genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group. The New England Journal Of Medicine, 339(14), 960–965.PubMedCrossRefGoogle Scholar
  45. Zipes, D. P., Camm, A. J., Borggrefe, M., Buxton, A. E., Chaitman, B., Fromer, M., et al. (2006). ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). Journal of the American College of Cardiology, 48(5), e247–e346.PubMedCrossRefGoogle Scholar
  46. Zvolensky, M. J., Eifert, G. H., Feldner, M. T., & Leen-Feldner, E. (2003). Heart-focused anxiety and chest pain in postangiography medical patients. Journal of Behavioral Medicine, 26(3), 197–209.PubMedCrossRefGoogle Scholar

Copyright information

© National Society of Genetic Counselors, Inc. 2011

Authors and Affiliations

  • Anniken Hamang
    • 1
    • 2
    • 7
  • Geir Egil Eide
    • 3
    • 4
  • Berit Rokne
    • 5
  • Karin Nordin
    • 5
    • 6
  • Cathrine Bjorvatn
    • 1
    • 2
  • Nina Øyen
    • 1
    • 2
  1. 1.Genetic Epidemiology Research Group, Department of Public Health and Primary Health CareUniversity of BergenBergenNorway
  2. 2.Center for Medical Genetics and Molecular MedicineHaukeland University HospitalBergenNorway
  3. 3.Centre for Clinical ResearchHaukeland University HospitalBergenNorway
  4. 4.Research Group on Lifestyle Epidemiology, Department of Public Health and Primary Health CareUniversity of BergenBergenNorway
  5. 5.Department of Public Health and Primary Health CareUniversity of BergenBergenNorway
  6. 6.Department of Public Health and Caring SciencesUppsala UniversityUppsalaSweden
  7. 7.Section of Medical GeneticsTrondheimNorway

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