Incremental value of the signal-averaged ECG for diagnosing arrhythmogenic cardiomyopathy

Published:October 13, 2022DOI:


      Arrhythmogenic right ventricular cardiomyopathy (ARVC) is currently diagnosed using a combination of clinical features, imaging, electrocardiography, and genetic investigations. An abnormal signal-averaged electrocardiogram (SAECG) is defined as a minor diagnostic criterion by the 2010 Task Force Criteria, but doubts remain about the value of this investigation.


      We evaluated the utility of the SAECG in diagnosing ARVC using the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry, a population representative registry of probands with ARVC and relatives, less influenced by referral bias.


      Probands with ARVC and family members from the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry underwent phenotype review. SAECG parameters were compared individually and in combination between those with varying degrees of ARVC severity and healthy controls (family members of probands with ARVC and unexplained sudden death, free of evidence of cardiac disease).


      A total of 196 patients with ARVC and 205 controls were included (mean age 44 ± 15 years; 186 of 401 men [46%]). SAECG abnormalities were seen in 83 of 205 controls (40%), 33 of 68 patients with ARVC and mild disease (51%), and 31 of 42 with severe disease (74%). The SAECG associated strongly with imaging abnormalities (major: odds ratio 3.0, 95% confidence interval 1.3–6.9; minor: odds ratio 3.5, 95% confidence interval 0.7–16.5) but not with other aspects of phenotype. Patients carrying pathogenic variants but with minimal phenotype had similar SAECGs to healthy controls (filtered QRS duration 111.2 ± 11.2 ms vs 111 ± 7.6 ms, P = .93; duration of low amplitude signals < 40 μV 32.3 ± 8.9 ms vs 34.2 ± 7.2 ms, P = .32; root mean square of the terminal 40 ms of the filtered QRS complex 43.1 ± 25.2 ms vs 38.2 ± 20.2 ms, P = .38).


      The SAECG appears to be a surrogate marker for structural abnormalities seen on imaging in those with ARVC. Great caution is required in interpreting SAECG findings in those without other corroborating evidence of an ARVC phenotype.


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        • Bosman L.P.
        • Te Riele A.
        Arrhythmogenic right ventricular cardiomyopathy: a focused update on diagnosis and risk stratification.
        Heart. 2022; 108: 90-97
        • Marcus F.I.
        • McKenna W.J.
        • Sherrill D.
        • et al.
        Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria.
        Eur Heart J. 2010; 31: 806-814
        • Bosman L.P.
        • Cadrin-Tourigny J.
        • Bourfiss M.
        • et al.
        Diagnosing arrhythmogenic right ventricular cardiomyopathy by 2010 Task Force Criteria: clinical performance and simplified practical implementation.
        Europace. 2020; 22: 787-796
        • Blomström-Lundqvist C.
        • Hirsch I.
        • Olsson S.B.
        • Edvardsson N.
        Quantitative analysis of the signal-averaged QRS in patients with arrhythmogenic right ventricular dysplasia.
        Eur Heart J. 1988; 9: 301-312
        • Huang Z.
        • Patel C.
        • Li W.
        • et al.
        Role of signal-averaged electrocardiograms in arrhythmic risk stratification of patients with Brugada syndrome: a prospective study.
        Heart Rhythm. 2009; 6: 1156-1162
        • Marcus F.I.
        • Zareba W.
        • Sherrill D.
        Evaluation of the normal values for signal-averaged electrocardiogram.
        J Cardiovasc Electrophysiol. 2007; 18: 231-233
        • Krahn A.D.
        • Healey J.S.
        • Gerull B.
        • et al.
        The Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry: rationale, design, and preliminary recruitment.
        Can J Cardiol. 2016; 32: 1396-1401
        • Denham N.C.
        • Pearman C.M.
        • Ding W.Y.
        • et al.
        Systematic re-evaluation of SCN5A variants associated with Brugada syndrome.
        J Cardiovasc Electrophysiol. 2019; 30: 118-127
        • Richards S.
        • Aziz N.
        • Bale S.
        • et al.
        Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology.
        Genet Med. 2015; 17: 405-424
        • Corrado D.
        • van Tintelen P.J.
        • McKenna W.J.
        • et al.
        Arrhythmogenic right ventricular cardiomyopathy: evaluation of the current diagnostic criteria and differential diagnosis.
        Eur Heart J. 2020; 41: 1414-1429
        • Corrado D.
        • Perazzolo Marra M.
        • Zorzi A.
        • et al.
        Diagnosis of arrhythmogenic cardiomyopathy: the Padua criteria.
        Int J Cardiol. 2020; 319: 106-114
        • Oselladore L.
        • Nava A.
        • Buja G.
        • et al.
        Signal-averaged electrocardiography in familial form of arrhythmogenic right ventricular cardiomyopathy.
        Am J Cardiol. 1995; 75: 1038-1041
        • Turrini P.
        • Angelini A.
        • Thiene G.
        • et al.
        Late potentials and ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy.
        Am J Cardiol. 1999; 83: 1214-1219
        • Kamath G.S.
        • Zareba W.
        • Delaney J.
        • et al.
        Value of the signal-averaged electrocardiogram in arrhythmogenic right ventricular cardiomyopathy/dysplasia.
        Heart Rhythm. 2011; 8: 256-262
        • Marstrand P.
        • Axelsson A.
        • Thune J.J.
        • et al.
        Late potentials and their correlation with ventricular structure in patients with ventricular arrhythmias.
        Pacing Clin Electrophysiol. 2017; 40: 1466-1471
        • Jongman J.K.
        • Zaidi A.
        • Muggenthaler M.
        • Sharma S.
        Relationship between echocardiographic right-ventricular dimensions and signal-averaged electrocardiogram abnormalities in endurance athletes.
        Europace. 2015; 17: 1441-1448
        • Pearman C.M.
        • Walia J.
        • Alqarawi W.
        • et al.
        The clinical utility of procainamide-induced late potentials on the signal averaged ECG.
        Pacing Clin Electrophysiol. 2021; 44: 2046-2053

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