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Current Cardiology Reviews

Editor-in-Chief

ISSN (Print): 1573-403X
ISSN (Online): 1875-6557

Review Article

Epsilon Waves as an Extreme Form of Depolarization Delay: Focusing on the Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Author(s): Pyotr G. Platonov* and Anneli Svensson

Volume 17, Issue 1, 2021

Published on: 10 August, 2020

Page: [17 - 23] Pages: 7

DOI: 10.2174/1573403X16666200810105029

open access plus

Abstract

Revision of the Task Force diagnostic criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), in 2010, has increased the sensitivity for the diagnosis of early and familial forms of the disease. Epsilon wave (EW) is a major diagnostic criterion in the context of ARVC/D, however, it remains unquantifiable and therefore, may leave room for substantial subjective interpretation, thus, explaining the existing high inter-observer variability in the assessment of EW. EW, when present, coexists with other disease characteristics, which are sufficient for ARVC/D diagnosis, making EW generally not required for ARVC/D diagnosis. Nevertheless, EW remains an important part of the electrocardiographic phenotype of ARVC/D that may be useful in planning diagnostic work-up, which needs to be recognized.

Keywords: Epsilon wave, arrhythmogenic right ventricular cardiomyopathy/dysplasia, electrocardiography, task force criteria, depolarization delay, ECG.

Graphical Abstract
[1]
Fontaine G, Guiraudon G, Frank R, et al. Simulation studies and epicardial mapping in ventricular tachycardia: Study of mechanisms and selection for surgery. In H. Kulbertus (ed.). Re-entrant Arrhythmias. Mechanisms and Treatment. Lancaster, UK: MTP Pub. 1977;pp. 334–350.
[2]
Li GL, Saguner AM, Fontaine GH, Frank R. Epsilon waves: Milestones in the discovery and progress. Ann Noninvasive Electrocardiol 2018; 23(6): e12571.
[http://dx.doi.org/10.1111/anec.12571] [PMID: 29978588]
[3]
Hurst JW. Naming of the waves in the ECG, with a brief account of their genesis. Circulation 1998; 98(18): 1937-42.
[http://dx.doi.org/10.1161/01.CIR.98.18.1937] [PMID: 9799216]
[4]
Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: A report of 24 adult cases. Circulation 1982; 65(2): 384-98.
[http://dx.doi.org/10.1161/01.CIR.65.2.384] [PMID: 7053899]
[5]
Fontaine G, Fontaliran F. Arrhythmogenic right ventricular dysplasia masquerading as dilated cardiomyopathy. Am J Cardiol 1999; 84(9): 1143.
[PMID: 10569693]
[6]
Tanawuttiwat T, Te Riele AS, Philips B, et al. Electroanatomic correlates of depolarization abnormalities in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Cardiovasc Electrophysiol 2016; 27(4): 443-52.
[http://dx.doi.org/10.1111/jce.12925] [PMID: 26757204]
[7]
Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: Proposed modification of the task force criteria. Circulation 2010; 121(13): 1533-41.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.108.840827] [PMID: 20172911]
[8]
Fontaine G, Chen HS. Arrhythmogenic right ventricular dysplasia back in force. Am J Cardiol 2014; 113(10): 1735-9.
[http://dx.doi.org/10.1016/j.amjcard.2014.03.001] [PMID: 24792741]
[9]
Marcus FI. Electrocardiographic features of inherited diseases that predispose to the development of cardiac arrhythmias, long QT syndrome, arrhythmogenic right ventricular cardiomyopathy/dysplasia, and Brugada syndrome. J Electrocardiol 2000; 33(Suppl.): 1-10.
[http://dx.doi.org/10.1054/jelc.2000.20360] [PMID: 11265707]
[10]
Steriotis AK, Bauce B, Daliento L, et al. Electrocardiographic pattern in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 2009; 103(9): 1302-8.
[http://dx.doi.org/10.1016/j.amjcard.2009.01.017] [PMID: 19406276]
[11]
Marcus FI, Abidov A. Arrhythmogenic right ventricular cardiomyopathy 2012: Diagnostic challenges and treatment. J Cardiovasc Electrophysiol 2012; 23(10): 1149-53.
[http://dx.doi.org/10.1111/j.1540-8167.2012.02412.x] [PMID: 22909229]
[12]
You CC, Tseng YT, Hsieh MH. An epsilon wave in arrhythmogenic right ventricular cardiomyopathy/dysplasia. Int J Cardiol 2007; 119(2): e63-4.
[http://dx.doi.org/10.1016/j.ijcard.2007.02.051] [PMID: 17462761]
[13]
Nasir K, Bomma C, Tandri H, et al. Electrocardiographic features of arrhythmogenic right ventricular dysplasia/cardiomyopathy according to disease severity: A need to broaden diagnostic criteria. Circulation 2004; 110(12): 1527-34.
[http://dx.doi.org/10.1161/01.CIR.0000142293.60725.18] [PMID: 15381658]
[14]
Fontaine G, Fontaliran F, Hébert JL, et al. Arrhythmogenic right ventricular dysplasia. Annu Rev Med 1999; 50: 17-35.
[http://dx.doi.org/10.1146/annurev.med.50.1.17] [PMID: 10073261]
[15]
Quarta G, Ward D, Tomé Esteban MT, et al. Dynamic electrocardiographic changes in patients with arrhythmogenic right ventricular cardiomyopathy. Heart 2010; 96(7): 516-22.
[http://dx.doi.org/10.1136/hrt.2009.182949] [PMID: 20350987]
[16]
Gottschalk B, Gysel M, Barbosa-Barros R, et al. The use of fontaine leads in the diagnosis of arrhythmogenic right ventricular dysplasia. Ann Noninvasive Electrocardiol 2014; 19(3): 279-84.
[http://dx.doi.org/10.1111/anec.12153] [PMID: 24597934]
[17]
Peters S, Trümmel M. Diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy: Value of standard ECG revisited. Ann Noninvasive Electrocardiol 2003; 8(3): 238-45.
[http://dx.doi.org/10.1046/j.1542-474X.2003.08312.x] [PMID: 14510660]
[18]
Kenigsberg DN, Kalahasty G, Grizzard JD, Wood MA, Ellenbogen KA. Images in cardiovascular medicine. Intracardiac correlate of the epsilon wave in a patient with arrhythmogenic right ventricular dysplasia. Circulation 2007; 115(21): e538-9.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.106.685594] [PMID: 17533186]
[19]
Bainbridge MN, Li L, Tan Y, Cheong BY, Marian AJ. Identification of established arrhythmogenic right ventricular cardiomyopathy mutation in a patient with the contrasting phenotype of hypertrophic cardiomyopathy. BMC Med Genet 2017; 18(1): 24.
[http://dx.doi.org/10.1186/s12881-017-0385-8] [PMID: 28253841]
[20]
Kiès P, Bootsma M, Bax JJ, et al. Serial reevaluation for ARVD/C is indicated in patients presenting with left bundle branch block ventricular tachycardia and minor ECG abnormalities. J Cardiovasc Electrophysiol 2006; 17(6): 586-93.
[http://dx.doi.org/10.1111/j.1540-8167.2006.00442.x] [PMID: 16836703]
[21]
Platonov PG, Calkins H, Hauer RN, et al. High interobserver variability in the assessment of epsilon waves: Implications for diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. Heart Rhythm 2016; 13(1): 208-16.
[http://dx.doi.org/10.1016/j.hrthm.2015.08.031] [PMID: 26304715]
[22]
Wang J, Yang B, Chen H, et al. Epsilon waves detected by various electrocardiographic recording methods: In patients with arrhythmogenic right ventricular cardiomyopathy. Tex Heart Inst J 2010; 37(4): 405-11.
[PMID: 20844612]
[23]
Li GL, Saguner AM, Akdis D, Fontaine GH. Value of a novel 16-lead High-Definition ECG machine to detect conduction abnormalities in structural heart disease. Pacing Clin Electrophysiol 2018; 41(6): 643-55.
[http://dx.doi.org/10.1111/pace.13338] [PMID: 29603270]
[24]
Turrini P, Corrado D, Basso C, Nava A, Bauce B, Thiene G. Dispersion of ventricular depolarization-repolarization: A noninvasive marker for risk stratification in arrhythmogenic right ventricular cardiomyopathy. Circulation 2001; 103(25): 3075-80.
[http://dx.doi.org/10.1161/01.CIR.103.25.3075] [PMID: 11425771]
[25]
Fontaine GH, Duthoit G, Li G, Andreoletti L, Gandjbakhch E, Frank R. Epsilon wave on an electronic loop in a case of arrhythmogenic right ventricular dysplasia with myocarditis: An updated definition of the Epsilon wave. Europace 2017; 19(7): 1084-90.
[http://dx.doi.org/10.1093/europace/euw320] [PMID: 28062531]
[26]
Marcus FI. Epsilon waves aid in the prognosis and risk stratification of patients with ARVC/D. J Cardiovasc Electrophysiol 2015; 26(11): 1211-2.
[http://dx.doi.org/10.1111/jce.12775] [PMID: 26249852]
[27]
Protonotarios A, Anastasakis A, Tsatsopoulou A, et al. Clinical significance of epsilon waves in arrhythmogenic cardiomyopathy. J Cardiovasc Electrophysiol 2015; 26(11): 1204-10.
[http://dx.doi.org/10.1111/jce.12755] [PMID: 26183028]
[28]
Wu S, Wang P, Hou Y, Yang P, Xiao Y, Zhan X. Epsilon wave in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Pacing Clin Electrophysiol 2009; 32(1): 59-63.
[http://dx.doi.org/10.1111/j.1540-8159.2009.02176.x] [PMID: 19140914]
[29]
Jaoude SA, Leclercq JF, Coumel P. Progressive ECG changes in arrhythmogenic right ventricular disease. Evidence for an evolving disease. Eur Heart J 1996; 17(11): 1717-22.
[http://dx.doi.org/10.1093/oxfordjournals.eurheartj.a014756] [PMID: 8922921]
[30]
Alter P, Grimm W. Pronounced epsilon waves in arrhythmogenic right ventricular dysplasia. J Cardiovasc Electrophysiol 2004; 15(2): 248.
[http://dx.doi.org/10.1046/j.1540-8167.2004.03450.x] [PMID: 15028061]
[31]
Sajeev CG, Jayakumar TG, Krishnan MN, Venugopal K. Epsilon wave. Int J Cardiol 2004; 93(2-3): 315.
[http://dx.doi.org/10.1016/S0167-5273(03)00167-0] [PMID: 14975570]
[32]
Santucci P, Morton J, Picken M, Wilber D. Electroanatomic mapping of the right ventricle in a patient with a giant epsilon wave, ventricular tachycardia, and cardiac sarcoidosis. J Cardiovasc Electrophysiol 2004; 15: 1091e4.
[33]
Zorio E, Arnau MA, Rueda J, et al. The presence of epsilon waves in a patient with acute right ventricular infarction. Pacing Clin Electrophysiol 2005; 28(3): 245-7.
[http://dx.doi.org/10.1111/j.1540-8159.2005.40021.x] [PMID: 15733188]
[34]
George BA, Ko JM, Lensing FD, Kuiper JJ, Roberts WC. “Repaired” tetralogy of fallot mimicking arrhythmogenic right ventricular cardiomyopathy (another phenocopy). Am J Cardiol 2011; 108(2): 326-9.
[http://dx.doi.org/10.1016/j.amjcard.2011.03.042] [PMID: 21545987]
[35]
Uhl HS. A previously undescribed congenital malformation of the heart: Almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 1952; 91(3): 197-209.
[PMID: 12978573]
[36]
Letsas KP, Efremidis M, Weber R, et al. Epsilon-like waves and ventricular conduction abnormalities in subjects with type 1 ECG pattern of Brugada syndrome. Heart Rhythm 2011; 8(6): 874-8.
[http://dx.doi.org/10.1016/j.hrthm.2011.01.043] [PMID: 21315837]
[37]
Yu J, Hu J, Dai X, et al. SCN5A mutation in Chinese patients with arrhythmogenic right ventricular dysplasia. Herz 2014; 39(2): 271-5.
[http://dx.doi.org/10.1007/s00059-013-3998-5] [PMID: 24317018]
[38]
Ozeke O, Aras D, Cay S, Ozcan F, Acar B, Topaloglu S. Ajmaline-induced Epsilon wave: Its role is not only for diagnosis but also for risk stratification. Int J Cardiol 2018; 264: 99.
[http://dx.doi.org/10.1016/j.ijcard.2018.03.046] [PMID: 29776580]
[39]
Caldwell J, Redfearn D, Chiale PA, Baranchuk A. Ablation-induced epsilon wave. Heart Rhythm 2013; 10(11): 1737-8.
[http://dx.doi.org/10.1016/j.hrthm.2012.08.038] [PMID: 23102627]
[40]
Hoogendijk MG. Diagnostic dilemmas: Overlapping features of brugada syndrome and arrhythmogenic right ventricular cardiomyopathy. Front Physiol 2012; 3: 144.
[http://dx.doi.org/10.3389/fphys.2012.00144] [PMID: 22654761]
[41]
Cox MG, Nelen MR, Wilde AA, et al. Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Toward improvement of diagnostic ECG criteria. J Cardiovasc Electrophysiol 2008; 19(8): 775-81.
[http://dx.doi.org/10.1111/j.1540-8167.2008.01140.x] [PMID: 18373594]
[42]
Cortez D, Svensson A, Carlson J, et al. The S-wave angle identifies arrhythmogenic right ventricular cardiomyopathy in patients with electrocardiographically concealed disease phenotype. J Electrocardiol 2018; 51(6): 1003-8.
[http://dx.doi.org/10.1016/j.jelectrocard.2018.08.009] [PMID: 30497719]
[43]
Tschabrunn CM, Haqqani HM, Santangeli P, Zado ES, Marchlinski FE. 12-lead electrocardiogram to localize region of abnormal electroanatomic substrate in arrhythmogenic right ventricular cardiomyopathy. JACC Clin Electrophysiol 2017; 3(7): 654-65.
[http://dx.doi.org/10.1016/j.jacep.2017.01.009] [PMID: 29759533]
[44]
Turrini P, Corrado D, Basso C, Nava A, Thiene G. Non-invasive risk stratification in arrhythmogenic right ventricular cardiomyopathy. Ann Noninvasive Electrocardiol 2003; 8(2): 161-9.
[http://dx.doi.org/10.1046/j.1542-474X.2003.08212.x] [PMID: 12848799]
[45]
Gallo C, Blandino A, Giustetto C, et al. Arrhythmogenic right ventricular cardiomyopathy: ECG progression over time and correlation with long-term follow-up. J Cardiovasc Med (Hagerstown) 2016; 17(6): 418-24.
[http://dx.doi.org/10.2459/JCM.0000000000000354] [PMID: 27119598]
[46]
Corrado D, Calkins H, Link MS, et al. Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation 2010; 122(12): 1144-52.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.109.913871] [PMID: 20823389]
[47]
Bhonsale A, James CA, Tichnell C, et al. Risk stratification in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. Circ Arrhythm Electrophysiol 2013; 6(3): 569-78.
[http://dx.doi.org/10.1161/CIRCEP.113.000233] [PMID: 23671136]
[48]
Link MS, Laidlaw D, Polonsky B, et al. Ventricular arrhythmias in the North American multidisciplinary study of ARVC: predictors, characteristics, and treatment. J Am Coll Cardiol 2014; 64(2): 119-25.
[http://dx.doi.org/10.1016/j.jacc.2014.04.035] [PMID: 25011714]
[49]
Platonov PG, Haugaa KH, Bundgaard H, et al. Primary prevention of sudden cardiac death with implantable cardioverter-defibrillator therapy in patients with arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 2019; 123(7): 1156-62.
[http://dx.doi.org/10.1016/j.amjcard.2018.12.049] [PMID: 30678832]
[50]
Cadrin-Tourigny J, Bosman LP, Nozza A, et al. A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. Eur Heart J 2019; 40(23): 1850-8.
[http://dx.doi.org/10.1093/eurheartj/ehz103] [PMID: 30915475]

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