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Observational Study
. 2025 Mar 18;14(6):e037544.
doi: 10.1161/JAHA.124.037544. Epub 2025 Mar 7.

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

Aleksei A Savelev  1 , Eivind W Aabel  2   3 , Anneli Svensson  4 , Pia Dahlberg  5 , Alex Hørby Christensen  6 , Trine Madsen  7 , Henning Bundgaard  8   9 , Tiina Heliö  10 , Aevar Ulfarsson  1 , Thor Edvardsen  2   3 , Jesper H Svendsen  8   9 , Henrik K Jensen  11   12 , Kristina H Haugaa  2   3 , Pyotr G Platonov  1
Affiliations
Observational Study

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

Aleksei A Savelev et al. J Am Heart Assoc. .

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 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.

Keywords: ARVC; arrhythmogenic cardiomyopathy; arrhythmogenic right ventricular cardiomyopathy; signal‐averaged ECG; ventricular late potentials.

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Conflict of interest statement

Jesper H. Svendsen reports to be a member a Medtronic advisory board; Tiina Heliö is board member of ERN GuardHeart as the representative of Helsinki University Hospital, has research Collaboration with Blueprint Genetics Quest, and received Bristol Myers Squibb lecturer, member of Advisory Board fees; Aevar Ulfarsson received speaker fees from Pfizer, Abbott, and Orion pharma; Jesper H. Svendsen received research grants (institutional) from Medtronic, speaker fee from Medtronic and fee for being a member of advisory board (Medtronic); Henrik K. Jensen received lecture fees from Abbott Denmark, Amgen Denmark, and Biosense Webster Europe; Kristina H. Haugaa has received speakers' honoraria from Bristol Myers Squibb; Pyotr G. Platonov is a member of Advisory Board at Tenaya Therapeutics and received speaker fees from Pfizer. The remaining authors have no disclosures to report.

Figures

Figure 1
Figure 1. Study design and structure.
ARVC indicates arrhythmogenic right ventricular cardiomyopathy; CMR, cardiac magnetic resonance; LP, ventricular late potentials; RV, right ventricular; SAECG, signal averaged ECG; TFC, Task Force Criteria; and VT, ventricular tachycardia. See the main text for the information of TFC and TFC‐LP scores counting.
Figure 2
Figure 2. ROC curves for SAECG parameters as predictors of RV structural abnormalities in patients with ARVC.
See Table 2 for C‐statistics values. ARVC indicates arrhythmogenic right ventricular cardiomyopathy; fQRSd, filtered QRS duration; LAS40, low amplitude signals; LGE, late gadolinium enhancement; ROC, receiver‐operator characteristics; RV, right ventricle; RVEDVI, right ventricular end‐diastolic volume index; RVEF, right ventricular ejection fraction; and SAECG, signal‐averaged electrocardiography.
Figure 3
Figure 3. Change in the diagnostic category from the conventional Task Force Criteria 2010 to the modified diagnostic scores that do not include either terminal activation delay or SAECG‐derived ventricular late potentials.
Only 1 patient with definite ARVC was to be reclassified if TAD was not counted, compared with 37 patients who would no longer fit into the definite diagnostic category, if the SAECG results were not considered. LP indicates ventricular late potentials; SAECG, signal‐averaged electrocardiography; TAD, terminal activationd delay; and TFC, Task Force Criteria.
Figure 4
Figure 4. Genotype‐specific ROC curve analysis of the association between SAECG parameters and ascertainment of clinical ARVC diagnosis by TFC2010 with SAECG results not accounted for in the diagnostic score (TFC‐LP, see the Methods section for more explanation).
ARVC indicates arrhythmogenic right ventricular cardiomyopathy; fQRSd, filtered QRS duration; LAS40, low amplitude signals; LP, ventricular late potentials; ROC, receiver‐operator characteristics; SAECG, signal‐averaged electrocardiography; TFC, Task Force Criteria.
See this image and copyright information in PMC

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