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Hereditary Arrhythmias

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Clinical Cardiac Electrophysiology in Clinical Practice

Part of the book series: In Clinical Practice ((ICP))

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Abstract

The development and proliferation of new genetic tests in recent decades have led to huge advancements in our understanding of the different pathophysiologic mechanisms underlying hereditary arrhythmias. Among the hereditary disorders that predispose to cardiac arrhythmias, the most concerning are those associated with ventricular tachyarrhythmias, including ventricular tachycardia and ventricular fibrillation. The phenotypic expression of affected individuals may vary, ranging from no symptoms at all, palpitations only, syncope, aborted cardiac arrest, or sudden cardiac death (SCD). SCD in the young has a tremendous impact on the families in which it occurs. These familial conditions can be divided into two major groups: cardiac ion channelopathies (i.e. without associated structural disease) and heritable cardiomyopathies (with associated structural heart disease). Both categories are monogenic disorders, in which the disease phenotype is brought about by a defect in a single gene. Most of these disorders exhibit autosomal dominant modes of inheritance, incomplete penetrance and variable expressivity.

This chapter reviews the current data regarding clinical and genetic aspects of the long-QT syndrome, Brugada syndrome, and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D); here we summarize their incidence, etiologies, diagnoses, mechanisms of disease, management and related technical aspects.

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Authors and Affiliations

  1. Cardiology Department, Rabin Medical Center, Petah Tikva, Israel

    Alon Barsheshet MD

  2. Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

    Alon Barsheshet MD & Ilan Goldenberg MD

  3. Cardiology Division, University of Rochester Medical Center, Rochester, New York

    Alon Barsheshet MD & Ilan Goldenberg MD

  4. Cardiology Department, The Leviev Heart Center, Sheba Medical Center, Tel Hashomer, Israel

    Ilan Goldenberg MD

Authors
  1. Alon Barsheshet MD
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  2. Ilan Goldenberg MD
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Correspondence to Alon Barsheshet MD .

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Editors and Affiliations

  1. Department of Cardiology, University of Rochester Medical Center, Rochester, New York, USA

    David T. Huang, MD

  2. Department of Cardiology, University of Rochester Medical Center, Rochester, New York, USA

    Travis Prinzi, MD

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Barsheshet, A., Goldenberg, I. (2015). Hereditary Arrhythmias. In: Huang, MD, D., Prinzi, MD, T. (eds) Clinical Cardiac Electrophysiology in Clinical Practice. In Clinical Practice. Springer, London. https://doi.org/10.1007/978-1-4471-5433-4_8

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