Key result
Brugada syndrome is increasingly associated with structural abnormalities such as fibrosis and reduced contractility, suggesting it may need to be classified as a cardiomyopathy rather than a purely electrical disease.
Why the study?
Although traditionally viewed as a purely electrical disease, growing evidence indicates Brugada syndrome involves structural abnormalities and sarcomeric gene mutations, raising the question of whether it should be classified as a cardiomyopathy.
Brugada syndrome exhibits structural and electromechanical abnormalities, suggesting it may need to be reclassified as a cardiomyopathy rather than a pure channelopathy.
Challenges channelopathy model in Brugada; leaves open reclassification as cardiomyopathy pending prospective validation.
Brugada syndrome (BrS) has been often described as a purely electrical disease. However, current dogma surrounding this concept has shifted to accept that BrS is associated with structural abnormalities. Brugada syndrome is now associated with epicardial surface and interstitial fibrosis, reduced gap junction expression, increased collagen, and reduced contractility. The ventricular arrhythmias observed in BrS have been linked to an arrhythmogenic substrate (AS) located rather consistently in the right ventricular outflow tract, sparking much debate as to the significance of this anatomical position. The size of the AS is dynamic and can be altered due to a number of factors. A larger AS is associated with reduced contractility, and this impaired mechanical function may be responsible for syncopal episodes in BrS patients in the absence of arrhythmic events. While BrS is generally regarded as a channelopathy, recent studies have now identified also mutations in genes encoding for sarcomeric proteins to be associated with BrS. Future studies should evaluate electromechanical coupling in BrS, including calcium handling and sarcomeric alterations, and evaluate whether BrS should be classified as a cardiomyopathy.
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Pappone et al. (2020) conducted a review in Brugada syndrome. Brugada syndrome is increasingly associated with structural abnormalities such as fibrosis and reduced contractility, suggesting it may need to be classified as a cardiomyopathy rather than a purely electrical disease.
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