Publication: Brugada syndrome: A comprehensive review of pathophysiological mechanisms and risk stratification strategies
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Issued Date
2020-02-01
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ISSN
23529067
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2-s2.0-85078013059
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Mahidol University
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SCOPUS
Bibliographic Citation
IJC Heart and Vasculature. Vol.26, (2020)
Suggested Citation
Ka Hou Christien Li, Sharen Lee, Chengye Yin, Tong Liu, Tachapong Ngarmukos, Giulio Conte, Gan Xin Yan, Raymond W. Sy, Konstantinos P. Letsas, Gary Tse Brugada syndrome: A comprehensive review of pathophysiological mechanisms and risk stratification strategies. IJC Heart and Vasculature. Vol.26, (2020). doi:10.1016/j.ijcha.2020.100468 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/53774
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Title
Brugada syndrome: A comprehensive review of pathophysiological mechanisms and risk stratification strategies
Other Contributor(s)
The Second Hospital of Tianjin Medical University
Sydney Medical School
School of Biological and Chemical Sciences Queen Mary University of London
Cardiocentro Ticino Foundation
Evagelismos Hospital
Xiamen University
Royal Prince Alfred Hospital
Mahidol University
Newcastle University, United Kingdom
Lankenau Institute for Medical Research
Li Ka Shing Institute of Health Sciences
Sydney Medical School
School of Biological and Chemical Sciences Queen Mary University of London
Cardiocentro Ticino Foundation
Evagelismos Hospital
Xiamen University
Royal Prince Alfred Hospital
Mahidol University
Newcastle University, United Kingdom
Lankenau Institute for Medical Research
Li Ka Shing Institute of Health Sciences
Abstract
© 2020 Brugada syndrome (BrS) is an inherited ion channel channelopathy predisposing to ventricular arrhythmias and sudden cardiac death. Originally believed to be predominantly associated with mutations in SCN5A encoding for the cardiac sodium channel, mutations of 18 genes other than SCN5A have been implicated in the pathogenesis of BrS to date. Diagnosis is based on the presence of a spontaneous or drug-induced coved-type ST segment elevation. The predominant electrophysiological mechanism underlying BrS remains disputed, commonly revolving around the three main hypotheses based on abnormal repolarization, depolarization or current-load match. Evidence from computational modelling, pre-clinical and clinical studies illustrates that molecular abnormalities found in BrS lead to alterations in excitation wavelength (λ), which ultimately elevates arrhythmic risk. A major challenge for clinicians in managing this condition is the difficulty in predicting the subset of patients who will suffer from life-threatening ventricular arrhythmic events. Several repolarization risk markers have been used thus far, but these neglect the contributions of conduction abnormalities in the form of slowing and dispersion. Indices incorporating both repolarization and conduction based on the concept of λ have recently been proposed. These may have better predictive values than the existing markers. Current treatment options include pharmacological therapy to reduce the occurrence of arrhythmic events or to abort these episodes, and interventions such as implantable cardioverter-defibrillator insertion or radiofrequency ablation of abnormal arrhythmic substrate.