Right ventricular electromechanical abnormalities in Brugada syndrome: is this a cardiomyopathy?

European Heart Journal Supplements : Journal of the European Society of Cardiology
Carlo PapponeGiuseppe Ciconte

Abstract

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.

References

Mar 3, 2004·Circulation·Raymond TukkieHanno L Tan
Sep 11, 2010·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Theano PapavassiliuMartin Borggrefe
May 21, 2011·Journal of the American College of Cardiology·William P McNairUNKNOWN Familial Cardiomyopathy Registry Research Group
May 8, 2013·Genesis : the Journal of Genetics and Development·J S BennettD M Roden
Jun 6, 2015·Circulation Research·Hugues AbrielJosé Jalife
Oct 30, 2015·Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology·Boris RudicTheano Papavassiliu
Feb 27, 2016·Biochimica Et Biophysica Acta·Elena Zaklyazminskaya, Sergei Dzemeshkevich
May 14, 2017·Circulation. Arrhythmia and Electrophysiology·Carlo PapponeVincenzo Santinelli
Aug 28, 2018·Frontiers in Physiology·Michelle M MonaskyLuigi Anastasia
Oct 20, 2018·Pediatric Emergency Care·David P FitzgeraldStephen M Schexnayder

❮ Previous
Next ❯

Citations

May 11, 2021·Frontiers in Cardiovascular Medicine·Michelle M MonaskyCarlo Pappone
Jun 15, 2021·Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology·Martijn H van der ReePieter G Postema
Jul 3, 2021·International Journal of Molecular Sciences·Cristina BallaPaola Imbrici

❮ Previous
Next ❯

Related Concepts

Related Feeds

Arrhythmia

Arrhythmias are abnormalities in heart rhythms, which can be either too fast or too slow. They can result from abnormalities of the initiation of an impulse or impulse conduction or a combination of both. Here is the latest research on arrhythmias.

Cardiomyopathy

Cardiomyopathy is a disease of the heart muscle, that can lead to muscular or electrical dysfunction of the heart. It is often an irreversible disease that is associated with a poor prognosis. There are different causes and classifications of cardiomyopathies. Here are the latest discoveries pertaining to this disease.

© 2022 Meta ULC. All rights reserved