The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy

The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy

Background: Electrocardiographic findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) have been limited to small studies. Objectives: The authors aimed to analyze the electrocardiogram (ECG) characteristics of ALVC, to correlate ECG with cardiac magnetic resonance and genetic data, and...

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Main Authors: Leonardo Calò, MD, Cinzia Crescenzi, MD, Andrea Di Marco, MD, PhD, Francesca Fanisio, MD, Fabiana Romeo, MD, Alessio Gargaro, MSc, Annamaria Martino, MD, PhD, Chiara Cappelletto, MD, Marco Merlo, MD, Mattia Targetti, MD, Elisabetta Toso, MD, Federica Toto, MD, Maria Beatrice Musumeci, MD, Giacomo Tini, MD, Michele Ciabatti, MD, Matteo Stefanini, MD, Stefano Canestrelli, MD, Elisa Fedele, MD, Chiara Lanzillo, MD, PhD, Armando Fusco, MD, PhD, Federica Carla Sangiuolo, MD, PhD, Cinzia Radesich, MD, Maria Perotto, MD, Maurizio Pieroni, MD, Ruggiero Mango, MD, PhD, Alessio Gasperetti, MD, PhD, Camillo Autore, MD, Michela Casella, MD, PhD, Antonio Dello Russo, MD, PhD, Davide Stolfo, MD, PhD, Mikael Laredo, MD, PhD, Estelle Gandjbakhch, MD, PhD, Maddalena Graziosi, MD, PhD, Elena Biagini, MD, PhD, Costantina Catalano, MD, Ludovica Barile, MD, Fabrizio Drago, MD, Marianna Cicenia, MD, Anwar Baban, MD, PhD, Gemma Pelargonio, MD, PhD, Maria Lucia Narducci, MD, Federica Re, MD, Giovanni Peretto, MD, Elena Paiotti, MD, Carles Diez Lopez, MD, Iacopo Olivotto, MD, Fiorenzo Gaita, MD, Gianfranco Sinagra, MD, Giuseppe Novelli, MD, PhD
Format: Article
Language:English
Published: Elsevier 2025-06-01
Series:JACC: Advances
Subjects:
arrhythmias
arrhythmogenic cardiomyopathy
cardiomyopathy
desmoplakin
desmosome
ECG
Online Access:http://www.sciencedirect.com/science/article/pii/S2772963X25001838
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Summary:Background: Electrocardiographic findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) have been limited to small studies. Objectives: The authors aimed to analyze the electrocardiogram (ECG) characteristics of ALVC, to correlate ECG with cardiac magnetic resonance and genetic data, and to evaluate its prognostic value. Methods: We reviewed data of 125 consecutive patients with ALVC (81.5% desmoplakin pathogenic/likely pathogenic variants). The composite endpoint of major arrhythmic events (MAEs) included sudden cardiac death, aborted sudden cardiac death, and appropriate implantable cardioverter-defibrillator shock. Predictors of MAE were evaluated with logistic regression. Results: ALVC showed distinct ECG signs, including left posterior fascicular block (LPFB) (13.6%), pathological Q waves (26.4%), R/S ratio in V1 ≥0.5 (26.4%), and SV1 + RV6 ≤12 mm and RI + RII ≤8 mm (44%). Fifteen (12%) patients had a normal ECG. MAE occurred in 35 patients (28%). In multivariable analysis, LPFB (OR: 4.7; 95% CI: 1.2-18.3), syncope (OR: 84.95; 95% CI: 14-496), transmural late gadolinium enhancement (OR: 9.95; 95% CI: 2.3-36), and right ventricular ejection fraction (OR: 0.92; 95% CI: 0.87-0.97) were the independent predictors of MAE. The model including these 4 variables achieved a remarkable predictive capability (area under the curve: 0.9). In the primary prevention scenario, with Cox regression, LPFB (HR: 3.98; 95% CI: 1.3-12.0), syncope (HR: 19.13; 95% CI: 5.8-63.0), and transmural late gadolinium enhancement (HR: 10.57; 95% CI: 2.9-38.0) were independent predictors of MAE. Conclusions: In ALVC, ECG is a valuable diagnostic tool and may have a relevant prognostic role, since LFPB is a strong and independent predictor of MAE.
ISSN:2772-963X

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