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Hypertrophic Cardiomyopathy

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Cardiac CT and MR for Adult Congenital Heart Disease

Abstract

Hypertrophic cardiomyopathy (HCM) is a common genetic cardiomyopathy caused by mutations in genes which encode for the myofilament protein components of the sarcomere or the z-disc (Maron et al., Heart Rhythm 9(1):57–63, 2012; Konno et al., Current Opin Cardiol 25(3):205–209, 2010; Judge, JAMA 302(22):2471–2476, 2009; Maron et al., Lancet 381:242–255, 2012). It has a prevalence of 1 in 500 in the general population and is a global disease affecting patients in all continents (Maron, Am J Med 116(1):63–65, 2004) and of both genders (Olivotto et al., J Am Coll Cardiol 46(3):480–487, 2005). It is the leading cause of sudden death in young people, with an annual mortality rate of 1 % (Maron, JAMA 287(10):1308–1320, 2002). Since its first description over 50 years ago, the pathophysiology of the disease is still incompletely understood (Teare, Br Heart J 20(1):1–8, 1958). The disease is associated with tremendous heterogeneity with regard to its presentation, phenotype, and prognosis. The diagnosis for HCM is usually made clinically after symptom onset or cardiac events, but may also be found after routine 12-lead electrocardiogram (ECG), heart murmur on cardiac exam, or in family screening of probands.

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Authors and Affiliations

  1. Division of Cardiology, Beth Israel Deaconess Medical Center, PERFUSE Study Group, 330 Brookline Avenue, East Campus SH 462, Boston, MA, 02215, USA

    Raymond H. M. Chan MD, MPH, FRCP(C), BScPhm

  2. Cardiac CT and MRI, Division of Cardiology, Hypertrophic Cardiomyopathy Center, Tufts University School of Medicine, Tufts Medical Center, 800 Washington St, #70, Boston, MA, 02111, USA

    Martin S. Maron MD

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Correspondence to Raymond H. M. Chan MD, MPH, FRCP(C), BScPhm .

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  1. Department of Radiology, USC University Hospital Keck School of Medicine, Los Angeles, California, USA

    Farhood Saremi

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Chan, R.H.M., Maron, M.S. (2014). Hypertrophic Cardiomyopathy. In: Saremi, F. (eds) Cardiac CT and MR for Adult Congenital Heart Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8875-0_16

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