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Arrhythmogenic Cardiomyopathy

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Clinical Cardiogenetics

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Abstract

Arrhythmogenic Cardiomyopathy (ACM) is a progressive heart muscle disease characterized histologically by fibrofatty replacement of ventricular myocardium. It is relatively rare, affecting approximately 1 in 1000 to 1 in 5000 people. Patients typically present with ventricular arrhythmias between the second and fourth decades of life. In early stages, ventricular arrhythmias originating from the right ventricle (RV) are most prominent, whereas usually in later stages, structural and functional abnormalities occur, eventually leading to heart failure. In all stages, ACM can be the cause of sudden death. However, sudden death most frequently occurs in adolescence, mainly in athletes. From autopsy studies, it is known that massive amounts of fibrofatty tissue can replace large parts of normal myocardium even in young teenagers (Fig. 6.1).

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Authors and Affiliations

  1. Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands

    Moniek G. P. J. Cox, Anneline S. J. M. te Riele & Richard N. W. Hauer

  2. Netherlands Heart Institute, Utrecht, The Netherlands

    Moniek G. P. J. Cox, Anneline S. J. M. te Riele & Richard N. W. Hauer

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  1. Moniek G. P. J. Cox
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  2. Anneline S. J. M. te Riele
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  3. Richard N. W. Hauer
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Editors and Affiliations

  1. Consultant Cardiologist at the OLVG Hospital, Amsterdam, The Netherlands

    Hubert F. Baars

  2. Utrecht University, Utrecht, The Netherlands

    Pieter A. F. M. Doevendans

  3. Amsterdam University Medical Center, Amsterdam, The Netherlands

    Arjan C. Houweling

  4. University Medical Center Utrecht, Utrecht, The Netherlands

    J. Peter van Tintelen

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Cox, M.G.P.J., te Riele, A.S.J.M., Hauer, R.N.W. (2020). Arrhythmogenic Cardiomyopathy. In: Baars, H.F., Doevendans, P.A.F.M., Houweling, A.C., van Tintelen, J.P. (eds) Clinical Cardiogenetics. Springer, Cham. https://doi.org/10.1007/978-3-030-45457-9_6

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