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

Charles Michael Pearman; David Lee; Brianna Davies; Habib Khan; Rafik Tadros; Julia Cadrin-Tourigny; Jason D. Roberts; Shubhayan Sanatani; Christopher Simpson; Paul Angaran; Simon Hansom; Erkan Ilhan; Colette Seifer; Martin Green; Martin Gardner; Mario Talajic; Zachary Laksman; Jeff S. Healey; Andrew Krahn

doi:10.1016/j.hrthm.2022.10.005

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

Charles Michael Pearman, David Lee, Brianna Davies, Habib Khan, Rafik Tadros, Julia Cadrin-Tourigny, Jason D. Roberts, Shubhayan Sanatani, Christopher Simpson, Paul Angaran, Simon Hansom, Erkan Ilhan, Colette Seifer, Martin Green, Martin Gardner, Mario Talajic, Zachary Laksman, Jeff S. Healey, Andrew Krahn

Division of Cardiovascular Sciences (L5)

Research output: Contribution to journal › Article › peer-review

Abstract

Background: 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.

Objective: 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.

Methods: 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).

Results: 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, confidence interval 1.3-6.9; minor: odds ratio 3.5, 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).

Conclusion: 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.

Original language	English
Pages (from-to)	224-230
Number of pages	7
Journal	Heart Rhythm
Volume	20
Issue number	2
Early online date	14 Oct 2022
DOIs	https://doi.org/10.1016/j.hrthm.2022.10.005
Publication status	Published - 1 Feb 2023

Keywords

Arrhythmogenic cardiomyopathy
Late potentials
SAECG
Sensitivity
Specificity

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1016/j.hrthm.2022.10.005

Cite this

Pearman, C. M., Lee, D., Davies, B., Khan, H., Tadros, R., Cadrin-Tourigny, J., Roberts, J. D., Sanatani, S., Simpson, C., Angaran, P., Hansom, S., Ilhan, E., Seifer, C., Green, M., Gardner, M., Talajic, M., Laksman, Z., Healey, J. S., & Krahn, A. (2023). Incremental value of the signal-averaged ECG for diagnosing arrhythmogenic cardiomyopathy. Heart Rhythm, 20(2), 224-230. https://doi.org/10.1016/j.hrthm.2022.10.005

@article{f8c3d2cb577647079fa41b9eb4009eb5,

title = "Incremental value of the signal-averaged ECG for diagnosing arrhythmogenic cardiomyopathy",

abstract = "Background: 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.Objective: 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.Methods: 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).Results: 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, confidence interval 1.3-6.9; minor: odds ratio 3.5, 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).Conclusion: 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.",

keywords = "Arrhythmogenic cardiomyopathy, Late potentials, SAECG, Sensitivity, Specificity",

author = "Pearman, {Charles Michael} and David Lee and Brianna Davies and Habib Khan and Rafik Tadros and Julia Cadrin-Tourigny and Roberts, {Jason D.} and Shubhayan Sanatani and Christopher Simpson and Paul Angaran and Simon Hansom and Erkan Ilhan and Colette Seifer and Martin Green and Martin Gardner and Mario Talajic and Zachary Laksman and Healey, {Jeff S.} and Andrew Krahn",

note = "Funding Information: Funding Sources: This work was supported by the Cardiology Academic Practice Plan at the University of British Columbia (Dr Laksman). The National Hearts in Rhythm Oragnization and Canadian Arrhythmogenic Right Ventricular Cardiomyopathy registries are funded by the Canadian Institutes of Health Research (Hearts in Rhythm Organization, RN380020-406814 and National Arrhythmogenic Right Ventricular Cardiomyopathy Registry, PJT-156229; Principal Investigator: Dr Krahn). Dr Krahn receives support from the Sauder Family and Heart and Stroke Foundation Chair in Cardiology (Vancouver, BC), the Paul Brunes Chair in Heart Rhythm Disorders (Vancouver, BC), and the Paul Albrechtsen Foundation (Winnipeg, MB). Publisher Copyright: {\textcopyright} 2022 Heart Rhythm Society",

year = "2023",

month = feb,

day = "1",

doi = "10.1016/j.hrthm.2022.10.005",

language = "English",

volume = "20",

pages = "224--230",

journal = "Heart Rhythm",

issn = "1547-5271",

publisher = "Elsevier BV",

number = "2",

}

Pearman, CM, Lee, D, Davies, B, Khan, H, Tadros, R, Cadrin-Tourigny, J, Roberts, JD, Sanatani, S, Simpson, C, Angaran, P, Hansom, S, Ilhan, E, Seifer, C, Green, M, Gardner, M, Talajic, M, Laksman, Z, Healey, JS & Krahn, A 2023, 'Incremental value of the signal-averaged ECG for diagnosing arrhythmogenic cardiomyopathy', Heart Rhythm, vol. 20, no. 2, pp. 224-230. https://doi.org/10.1016/j.hrthm.2022.10.005

TY - JOUR

T1 - Incremental value of the signal-averaged ECG for diagnosing arrhythmogenic cardiomyopathy

AU - Pearman, Charles Michael

AU - Lee, David

AU - Davies, Brianna

AU - Khan, Habib

AU - Tadros, Rafik

AU - Cadrin-Tourigny, Julia

AU - Roberts, Jason D.

AU - Sanatani, Shubhayan

AU - Simpson, Christopher

AU - Angaran, Paul

AU - Hansom, Simon

AU - Ilhan, Erkan

AU - Seifer, Colette

AU - Green, Martin

AU - Gardner, Martin

AU - Talajic, Mario

AU - Laksman, Zachary

AU - Healey, Jeff S.

AU - Krahn, Andrew

N1 - Funding Information: Funding Sources: This work was supported by the Cardiology Academic Practice Plan at the University of British Columbia (Dr Laksman). The National Hearts in Rhythm Oragnization and Canadian Arrhythmogenic Right Ventricular Cardiomyopathy registries are funded by the Canadian Institutes of Health Research (Hearts in Rhythm Organization, RN380020-406814 and National Arrhythmogenic Right Ventricular Cardiomyopathy Registry, PJT-156229; Principal Investigator: Dr Krahn). Dr Krahn receives support from the Sauder Family and Heart and Stroke Foundation Chair in Cardiology (Vancouver, BC), the Paul Brunes Chair in Heart Rhythm Disorders (Vancouver, BC), and the Paul Albrechtsen Foundation (Winnipeg, MB). Publisher Copyright: © 2022 Heart Rhythm Society

PY - 2023/2/1

Y1 - 2023/2/1

N2 - Background: 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.Objective: 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.Methods: 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).Results: 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, confidence interval 1.3-6.9; minor: odds ratio 3.5, 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).Conclusion: 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.

AB - Background: 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.Objective: 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.Methods: 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).Results: 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, confidence interval 1.3-6.9; minor: odds ratio 3.5, 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).Conclusion: 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.

KW - Arrhythmogenic cardiomyopathy

KW - Late potentials

KW - SAECG

KW - Sensitivity

KW - Specificity

U2 - 10.1016/j.hrthm.2022.10.005

DO - 10.1016/j.hrthm.2022.10.005

M3 - Article

SN - 1547-5271

VL - 20

SP - 224

EP - 230

JO - Heart Rhythm

JF - Heart Rhythm

IS - 2

ER -

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

Abstract

Keywords

UN SDGs

Access to Document

Fingerprint

Cite this