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.
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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
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
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.
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.
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.
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.
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.
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.
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
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.
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.
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.
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.
Elliott, P., & Spirito, P. (2008). Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice. Heart, 94(10), 1269–1275.
Fayers, P. M., & Sprangers, M. A. (2002). Understanding self-rated health. Lancet, 359(9302), 187–188.
Fulda, K. G., & Lykens, K. (2006). Ethical issues in predictive genetic testing: a public health perspective. Journal of Medical Ethics, 32(3), 143–147.
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.
Goldenberg, I., & Moss, A. J. (2008). Long QT Syndrome. Journal of the American College of Cardiology, 51(24), 2291–2300.
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.
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.
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.
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
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.
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.
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.
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.
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.
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.
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.
Maron, B. J. (2002). Hypertrophic cardiomyopathy: a systematic review. JAMA, 287(10), 1308–1320.
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.
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
Priori, S. G., Napolitano, C., & Schwartz, P. J. (1999). Low penetrance in the long-QT syndrome: clinical impact. Circulation, 99(4), 529–533.
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.
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.
Shiloh, S., Avdor, O., & Goodman, R. M. (1990). Satisfaction with genetic counseling: dimensions and measurement. American Journal of Medical Genetics, 37(4), 522–529.
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.
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.
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.
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.
Ware, J. E., Kosinki, M., & Gandek, B. (2000). SF-36 Health Survey: manual & interpretation guide (2nd ed.). Lincoln, Rhode Island: QualityMetric Inc.
Watkins, H., Ashrafian, H., & Redwood, C. (2011). Inherited cardiomyopathies. The New England Journal of Medicine, 364(17), 1643–1656.
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.
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.
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.
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.
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.
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Hamang, A., Eide, G.E., Rokne, B. et al. Predictors of Heart-Focused Anxiety in Patients Undergoing Genetic Investigation and Counseling of Long QT Syndrome or Hypertrophic Cardiomyopathy: A One Year Follow-up. J Genet Counsel 21, 72–84 (2012). https://doi.org/10.1007/s10897-011-9393-6
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DOI: https://doi.org/10.1007/s10897-011-9393-6