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Inherited Cardiac Muscle Disorders: Arrhythmogenic Right Ventricular Cardiomyopathy

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Cardiovascular Genetics and Genomics

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Abstract

Arrhythmogenic Cardiomyopathy (AC) is characterized by cardiomyocyte death with fibro-fatty repair, clinically manifesting with palpitations, syncope or cardiac arrest usually in adolescence or young adulthood. AC represents one of the major causes of sudden death in the young and athletes. The estimated prevalence of AC in the general population ranges from 1:2000 to 1:5000. AC affects more frequently males than females (up to 3:1), despite a similar prevalence of carrier status, and due to age-related penetrance becomes clinically overt most often in the second-fourth decade of life.

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Authors and Affiliations

  1. Department of Cardiac, Thoracic and Vascular Sciences, Pathological Anatomy-Cardiovascular Pathology, University of Padua Medical School, Padua, Italy

    Kalliopi Pilichou, Barbara Bauce, Gaetano Thiene & Cristina Basso

  2. Cardio-Thoracic and Vascular Sciences, Policlinico Universitario di Padova, Padova, Italy

    Kalliopi Pilichou, Barbara Bauce, Gaetano Thiene & Cristina Basso

Authors
  1. Kalliopi Pilichou
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  2. Barbara Bauce
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  3. Gaetano Thiene
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  4. Cristina Basso
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Correspondence to Kalliopi Pilichou .

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Editors and Affiliations

  1. Institute of Cancer & Genetics, University Hospital of Wales, Cardiff, United Kingdom

    Dhavendra Kumar

  2. Barts Heart Centre, St Bartholomew’s Hospital, London, United Kingdom

    Perry Elliott

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Pilichou, K., Bauce, B., Thiene, G., Basso, C. (2018). Inherited Cardiac Muscle Disorders: Arrhythmogenic Right Ventricular Cardiomyopathy. In: Kumar, D., Elliott, P. (eds) Cardiovascular Genetics and Genomics. Springer, Cham. https://doi.org/10.1007/978-3-319-66114-8_11

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