Abstract
Early repolarization (ER) is a common electrocardiographic finding characterized by elevation of the J point with notching or slurring on the electrocardiogram (ECG) followed by ST-segment elevation. Although this condition has been considered benign, Haïssaguerre et al. demonstrated that patients with J waves in the inferolateral leads were likely to be associated with idiopathic ventricular fibrillation (VF) and reported this entity as inferolateral early repolarization syndrome (ERS) in 2008. Brugada syndrome (BrS) is another clinical entity that causes sudden death due to VF in patients with apparently structurally normal hearts and is characterized by coved ST-segment elevation in the right precordial leads. ERS and BrS are considered to share a similar genetic background and to represent a continuous spectrum of phenotypic expression. However, the exact pathophysiological mechanisms underlying ERS and BrS remain unknown, and some clinical manifestations reportedly differ between these syndromes. In this chapter, we introduce our current understanding of ERS and BrS.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Haïssaguerre M, Derval N, Sacher F, et al. Sudden cardiac arrest associated with early repolarization. N Engl J Med. 2008;358:2016–23.
Tikkanen JT, Anttonen O, Junttila MJ, et al. Long-term outcome associated with early repolarization on electrocardiography. N Engl J Med. 2009;361:2529–37.
Rosso R, Kogan E, Belhassen B, et al. J-point elevation in survivors of primary ventricular fibrillation and matched control subjects: incidence and clinical significance. J Am Coll Cardiol. 2008;52:1231–8.
Haruta D, Matsuo K, Tsuneto A, et al. Incidence and prognostic value of early repolarization pattern in the 12–lead electrocardiogram. Circulation. 2011;123:2931–7.
Wilde AA, Antzelevitch C, Borggrefe M, et al. Study Group on the Molecular Basis of Arrhythmias of the European Society of Cardiology. Proposed diagnostic criteria for the Brugada syndrome: consensus report. Circulation. 2002;106:2514–9.
Antzelevitch C, Brugada P, Borgreffe M, et al. Brugada syndrome; report of the second consensus conference; endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005;111:659–70.
Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. 1992;20:1391–6.
Antzelevitch C, Yan GX. J wave syndromes. Heart Rhythm. 2010;7:549–58.
Haïssaguerre M, Sacher F, Nogami A, et al. Characteristics of recurrent ventricular fibrillation associated with inferolateral early repolarization. Role of drug therapy. J Am Coll Cardiol. 2009;53:612–9.
Aizawa Y, Sato A, Watanabe H, et al. Dynamicity of the J-wave in idiopathic ventricular fibrillation with a special reference to pause-dependent augmentation of the J-wave. J Am Coll Cardiol. 2012;59:1948–53.
Ohgo T, Okamura H, Noda T, et al. Acute and chronic management in patients with Brugada syndrome associated with electrical storm of ventricular fibrillation. Heart Rhythm. 2007;4:695–700.
Gourraud JB, Le Scouarnec S, Sacher F, et al. Identification of large families in early repolarization syndrome. J Am Coll Cardiol. 2013;61:164–72.
Haïssaguerre M, Chatel S, Sacher F, et al. Ventricular fibrillation with prominent early repolarization associated with a rare variant of KCNJ8/KATP channel. J Cardiovasc Electrophysiol. 2009;20:93–8.
Watanabe H, Nogami A, Ohkubo K, et al. Electrocardiographic characteristics and SCN5A mutations in idiopathic ventricular fibrillation associated with early repolarization. Circ Arrhythm Electrophysiol. 2011;4:874–81.
Medeiros-Domingo A, Tan BH, Crotti L, et al. Gain-of-function mutation S422L in the KCNJ8-encoded cardiac K(ATP) channel Kir6.1 as a pathogenic substrate for J-wave syndromes. Heart Rhythm. 2010;7:1466–71.
Barajas-Martínez H, Hu D, Ferrer T, et al. Molecular genetic and functional association of Brugada and early repolarization syndromes with S422L missense mutation in KCNJ8. Heart Rhythm. 2012;9:548–55.
Burashnikov E, Pfeiffer R, Barajas-Martinez H, et al. Mutations in the cardiac L-type calcium channel associated with inherited J-wave syndromes and sudden cardiac death. Heart Rhythm. 2010;7:1872–82.
Hu D, Barajas-Martinez H, Pfeiffer R, et al. Mutations in SCN10A are responsible for a large fraction of cases of Brugada syndrome. J Am Coll Cardiol. 2014;64:66–79.
Chen Q, Kirsch GE, Zhang D, et al. Genetic basis and molecular mechanisms for idiopathic ventricular fibrillation. Nature. 1998;392:293–6.
Antzelevitch C, Pollevick GD, Cordeiro JM, et al. Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST segment elevation, short QT intervals, and sudden cardiac death. Circulation. 2007;115:442–9.
Mahida S, Derval N, Sacher F, et al. Role of electrophysiological studies in predicting risk of ventricular arrhythmia in early repolarization syndrome. J Am Coll Cardiol. 2015;65:151–9.
Kawata H, Noda T, Yamada Y, et al. Effect of sodium-channel blockade on early repolarization in inferior/lateral leads in patients with idiopathic ventricular fibrillation and Brugada syndrome. Heart Rhythm. 2012;9:77–83.
Roten L, Derval N, Sacher F, et al. Ajimaline attenuates electrocardiogram characteristics of inferolateral early repolarization. Heart Rhythm. 2012;9:232–9.
Nam GB, Ko KH, Kim J, et al. Mode of onset of ventricular fibrillation in patients with early repolarization pattern vs. Brugada syndrome. Eur Heart J. 2010;31:330–9.
Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013;10:1932–63.
Miyamoto K, Yokokawa M, Tanaka K, et al. Diagnostic and prognostic value of a type 1 Brugada electrocardiogram at higher (third or second) V1 to V2 recording in men with Brugada syndrome. Am J Cardiol. 2007;99:53–7.
Govindan M, Batchvarov VN, Raju H, et al. Utility of high and standard right precordial leads during ajmaline testing for the diagnosis of Brugada syndrome. Heart. 2010;96:1904–8.
Savastano S, Rordorf R, Vicentini A, et al. A comprehensive electrocardiographic, molecular, and echocardiographic study of Brugada syndrome: validation of the 2013 diagnostic criteria. Heart Rhythm. 2014;11:1176–83.
Kamakura T, Wada M, Nakajima I, et al. Significance of electrocardiogram recording in high intercostal spaces in patients with early repolarization syndrome. Eur Heart J. 2016;37:630–7.
Kamakura S, Ohe T, Nakazawa K, et al. Brugada syndrome investigators in Japan. Long-term prognosis of probands with Brugada-pattern ST-elevation in leads V1-3. Circ Arrhythm Electrophysiol. 2009;2:495–503.
Kamakura T, Kawata H, Nakajima I, et al. Significance of non-type 1 anterior early repolarization in patients with inferolateral early repolarization syndrome. J Am Coll Cardiol. 2013;62:1610–8.
Kamakura T, Wada M, Ishibashi K, et al. Differences in the onset mode of ventricular tachyarrhythmia between patients with J wave in anterior leads and those with J wave in inferolateral leads. Heart Rhythm. 2016; doi:10.1016/j.hrthm.2016.11.027.
Wilde AA, Postema PG, Di Diego JM, et al. The pathophysiological mechanism underlying Brugada syndrome: depolarization versus repolarization. J Mol Cell Cardiol. 2010;49:543–53.
Nademanee K, Veerakul G, Chandanamattha P, et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation. 2011;123:1270–9.
Abe A, Ikeda T, Tsukada T, et al. Circadian variation of late potentials in idiopathic ventricular fibrillation associated with J waves: insights into alternative pathophysiology and risk stratification. Heart Rhythm. 2010;7:675–82.
Nakagawa M, Ezaki K, Miyazaki H, et al. Electrocardiographic characteristics of patients with false tendon: possible association of false tendon with J waves. Heart Rhythm. 2012;9:782–8.
Shimizu W, Matsuo K, Kokubo Y, et al. Sex hormone and gender difference: role of testosterone on male predominance in Brugada syndrome. J Cardiovasc Electrophysiol. 2007;18:415–21.
Junttila MJ, Tikkanen JT, Porthan K, et al. Relationship between testosterone level and early repolarization on 12-lead electrocardiograms in men. J Am Coll Cardiol. 2013;62:1633–4.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer Nature Singapore Pte Ltd.
About this chapter
Cite this chapter
Kamakura, T., Kamakura, S. (2018). ERS in Relation to Brugada Syndrome. In: Shimizu, W. (eds) Early Repolarization Syndrome. Springer, Singapore. https://doi.org/10.1007/978-981-10-3379-7_5
Download citation
DOI: https://doi.org/10.1007/978-981-10-3379-7_5
Published:
Publisher Name: Springer, Singapore
Print ISBN: 978-981-10-3378-0
Online ISBN: 978-981-10-3379-7
eBook Packages: MedicineMedicine (R0)