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Signal-Averaged ECG in the Diagnostic Workup for Arrhythmogenic Cardiomyopathy : Insights From the Nordic ARVC Registry

Savelev, Aleksei A LU orcid ; Aabel, Eivind W ; Svensson, Anneli LU ; Dahlberg, Pia LU ; Christensen, Alex Hørby ; Madsen, Trine ; Bundgaard, Henning ; Heliö, Tiina ; Ulfarsson, Aevar LU and Edvardsen, Thor , et al. (2025) In Journal of the American Heart Association 14(6). p.1-10
Abstract

BACKGROUND: The diagnostic role of signal-averaged ECG (SAECG) in arrhythmogenic right ventricular cardiomyopathy (ARVC) has lately been questioned. We assessed the value of SAECG-derived late ventricular potentials (LP) in ARVC diagnosis and its association with disease manifestations.

METHODS AND RESULTS: Patients with definite ARVC diagnosis or genotype-positive family members who underwent SAECG were included in register-based observational study (n=357, mean age 41 years, 47% female, 43% probands). LP and terminal activation duration (TAD) were defined by Task Force Criteria 2010. We assessed the association of TAD and LP with structural RV abnormalities and ventricular tachycardia (VT), defined as sustained VT, appropriate... (More)

BACKGROUND: The diagnostic role of signal-averaged ECG (SAECG) in arrhythmogenic right ventricular cardiomyopathy (ARVC) has lately been questioned. We assessed the value of SAECG-derived late ventricular potentials (LP) in ARVC diagnosis and its association with disease manifestations.

METHODS AND RESULTS: Patients with definite ARVC diagnosis or genotype-positive family members who underwent SAECG were included in register-based observational study (n=357, mean age 41 years, 47% female, 43% probands). LP and terminal activation duration (TAD) were defined by Task Force Criteria 2010. We assessed the association of TAD and LP with structural RV abnormalities and ventricular tachycardia (VT), defined as sustained VT, appropriate implantable cardioverter-defibrillator shock, aborted cardiac arrest, or sudden cardiac death, at diagnosis. LP were documented in 210 patients (59%) and abnormal TAD in 66 patients (18%). Each of the SAECG parameters was significantly associated with definite ARVC diagnosis in receiver-operator characteristics curve analysis with area under the curve between 0.67 and 0.74. Exclusion of SAECG from diagnostic workup led to reclassification of 37 patients (16%) from definite to borderline ARVC (13 probands, 9 of whom had prevalent VT). Ninety patients (25%) had history of VT. LP, but not TAD, were associated with VT (adjusted odds ratio [ORadj], 2.42 [95%CI, 1.07-5.48]). LP had lower specificity (72% versus 97%) but higher sensitivity (71% versus 25%) for association with RV structural abnormalities than TAD.

CONCLUSIONS: In the Nordic ARVC cohort SAECG-derived LP are associated with VT and structural RV abnormalities and were critical for ascertainment of ARVC diagnosis in 16% of patients with narrow QRS complexes, including 8% of all probands.

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publication status
published
subject
keywords
Humans, Arrhythmogenic Right Ventricular Dysplasia/diagnosis, Female, Male, Electrocardiography/methods, Registries, Adult, Middle Aged, Predictive Value of Tests, Tachycardia, Ventricular/diagnosis, Action Potentials, Scandinavian and Nordic Countries/epidemiology, ROC Curve, Death, Sudden, Cardiac/etiology
in
Journal of the American Heart Association
volume
14
issue
6
article number
e037544
pages
1 - 10
publisher
Wiley-Blackwell
external identifiers
  • pmid:40055861
  • scopus:105001222943
ISSN
2047-9980
DOI
10.1161/JAHA.124.037544
language
English
LU publication?
yes
id
6aaabb2c-7c5a-488b-bccc-564afc086b33
date added to LUP
2025-03-20 14:03:32
date last changed
2025-07-12 04:35:38
@article{6aaabb2c-7c5a-488b-bccc-564afc086b33,
  abstract     = {{<p>BACKGROUND: The diagnostic role of signal-averaged ECG (SAECG) in arrhythmogenic right ventricular cardiomyopathy (ARVC) has lately been questioned. We assessed the value of SAECG-derived late ventricular potentials (LP) in ARVC diagnosis and its association with disease manifestations.</p><p>METHODS AND RESULTS: Patients with definite ARVC diagnosis or genotype-positive family members who underwent SAECG were included in register-based observational study (n=357, mean age 41 years, 47% female, 43% probands). LP and terminal activation duration (TAD) were defined by Task Force Criteria 2010. We assessed the association of TAD and LP with structural RV abnormalities and ventricular tachycardia (VT), defined as sustained VT, appropriate implantable cardioverter-defibrillator shock, aborted cardiac arrest, or sudden cardiac death, at diagnosis. LP were documented in 210 patients (59%) and abnormal TAD in 66 patients (18%). Each of the SAECG parameters was significantly associated with definite ARVC diagnosis in receiver-operator characteristics curve analysis with area under the curve between 0.67 and 0.74. Exclusion of SAECG from diagnostic workup led to reclassification of 37 patients (16%) from definite to borderline ARVC (13 probands, 9 of whom had prevalent VT). Ninety patients (25%) had history of VT. LP, but not TAD, were associated with VT (adjusted odds ratio [ORadj], 2.42 [95%CI, 1.07-5.48]). LP had lower specificity (72% versus 97%) but higher sensitivity (71% versus 25%) for association with RV structural abnormalities than TAD.</p><p>CONCLUSIONS: In the Nordic ARVC cohort SAECG-derived LP are associated with VT and structural RV abnormalities and were critical for ascertainment of ARVC diagnosis in 16% of patients with narrow QRS complexes, including 8% of all probands.</p>}},
  author       = {{Savelev, Aleksei A and Aabel, Eivind W and Svensson, Anneli and Dahlberg, Pia and Christensen, Alex Hørby and Madsen, Trine and Bundgaard, Henning and Heliö, Tiina and Ulfarsson, Aevar and Edvardsen, Thor and Svendsen, Jesper H and Jensen, Henrik K and Haugaa, Kristina H and Platonov, Pyotr G}},
  issn         = {{2047-9980}},
  keywords     = {{Humans; Arrhythmogenic Right Ventricular Dysplasia/diagnosis; Female; Male; Electrocardiography/methods; Registries; Adult; Middle Aged; Predictive Value of Tests; Tachycardia, Ventricular/diagnosis; Action Potentials; Scandinavian and Nordic Countries/epidemiology; ROC Curve; Death, Sudden, Cardiac/etiology}},
  language     = {{eng}},
  month        = {{03}},
  number       = {{6}},
  pages        = {{1--10}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Journal of the American Heart Association}},
  title        = {{Signal-Averaged ECG in the Diagnostic Workup for Arrhythmogenic Cardiomyopathy : Insights From the Nordic ARVC Registry}},
  url          = {{http://dx.doi.org/10.1161/JAHA.124.037544}},
  doi          = {{10.1161/JAHA.124.037544}},
  volume       = {{14}},
  year         = {{2025}},
}