Inherited arrhythmia syndrome predisposing to sudden cardiac death

Inherited arrhythmia (IA) is one of the main causes of sudden cardiac death (SCD) in young people, and is reported to be a more prevalent cause of SCD in Asia than in Western countries. IAs are a group of genetic disorders caused by mutations in genes encoding cardiac ion channels, leading to electr...

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Bibliographic Details
Published in:The Korean journal of internal medicine 2021, 36(3), , pp.527-538
Main Authors: Kim, Yun Gi, Oh, Suk-Kyu, Choi, Ha Young, Choi, Jong-Il
Format: Article
Language:English
Subjects:
channelopathies
death, sudden, cardiac
genetic testing
inherited arrhythmia
precision medicine
Review
내과학
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Summary:Inherited arrhythmia (IA) is one of the main causes of sudden cardiac death (SCD) in young people, and is reported to be a more prevalent cause of SCD in Asia than in Western countries. IAs are a group of genetic disorders caused by mutations in genes encoding cardiac ion channels, leading to electrophysiological characteristics that often occur in the absence of structural abnormalities. Channelopathies, such as long QT syndrome and Brugada syndrome, carry a potential risk of life-threatening ventricular tachyarrhythmias that predispose to SCD, although early prediction and prevention of the risk remain challenging. Recent advances in genetic testing have facilitated risk stratification as well as a precise diagnosis for IA, despite ongoing debates about the implications. Herein, we provide epidemiological data, a pathophysiological overview, and the current clinical approach to IAs related to SCD. In addition, we review the general issues arising from genetic testing for IAs.
ISSN:1226-3303
2005-6648
DOI:10.3904/kjim.2020.481