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 to...
| Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2025 |
| País: | España |
| Institución: | Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya) |
| Repositorio: | Recercat. Dipósit de la Recerca de Catalunya |
| OAI Identifier: | oai:recercat.cat:2445/222225 |
| Acceso en línea: | https://hdl.handle.net/2445/222225 |
| Access Level: | acceso abierto |
| Palabra clave: | Miocardiopaties Desfibril·ladors cardioversors implantables Myocardiopathies Implantable cardioverter-defibrillators |
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The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular CardiomyopathyCalò, LeonardoCrescenzi, CinziaDi Marco, AndreaFanisio, FrancescaRomeo, FabianaGargaro, AlessioMartino, AnnamariaCappelletto, ChiaraMerlo, MarcoTargetti, MattiaToso, ElisabettaToto, FedericaMusumeci, Maria BeatriceTini, GiacomoCiabatti, MicheleStefanini, MatteoCanestrelli, StefanoFedele, ElisaLanzillo, ChiaraFusco, ArmandoSangiuolo, Federica CarlaRadesich, CinziaPerotto, MariaPieroni, MaurizioMango, RuggieroGasperetti, AlessioAutore, CamilloCasella, MichelaDello Russo, AntonioStolfo, DavideLaredo, MikaelGandjbakhch, EstelleGraziosi, MaddalenaBiagini, ElenaCatalano, CostantinaBarile, LudovicaDrago, FabrizioCicenia, MariannaBaban, AnwarPelargonio, GemmaNarducci, Maria LuciaRe, FedericaPeretto, GiovanniPaiotti, ElenaDíez López, CarlesOlivotto, IacopoGaita, FiorenzoSinagra, GianfrancoNovelli, GiuseppeMiocardiopatiesDesfibril·ladors cardioversors implantablesMyocardiopathiesImplantable cardioverter-defibrillatorsBACKGROUND 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. (JACC Adv. 2025;4:101766) (c) 2025 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY license (http://creativecommons. org/licenses/by/4.0/).Elsevier BV2025202520252025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion15 p.application/pdfhttps://hdl.handle.net/2445/222225Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))reponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésReproducció del document publicat a: https://doi.org/10.1016/j.jacadv.2025.101766JACC Advances, 2025, vol. 4, num. 6https://doi.org/10.1016/j.jacadv.2025.101766cc by (c) Calò, Leonardo et al, 2025http://creativecommons.org/licenses/by/3.0/es/info:eu-repo/semantics/openAccessoai:recercat.cat:2445/2222252026-05-29T05:05:01Z |
| dc.title.none.fl_str_mv |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy |
| title |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy |
| spellingShingle |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy Calò, Leonardo Miocardiopaties Desfibril·ladors cardioversors implantables Myocardiopathies Implantable cardioverter-defibrillators |
| title_short |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy |
| title_full |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy |
| title_fullStr |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy |
| title_full_unstemmed |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy |
| title_sort |
The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy |
| dc.creator.none.fl_str_mv |
Calò, Leonardo Crescenzi, Cinzia Di Marco, Andrea Fanisio, Francesca Romeo, Fabiana Gargaro, Alessio Martino, Annamaria Cappelletto, Chiara Merlo, Marco Targetti, Mattia Toso, Elisabetta Toto, Federica Musumeci, Maria Beatrice Tini, Giacomo Ciabatti, Michele Stefanini, Matteo Canestrelli, Stefano Fedele, Elisa Lanzillo, Chiara Fusco, Armando Sangiuolo, Federica Carla Radesich, Cinzia Perotto, Maria Pieroni, Maurizio Mango, Ruggiero Gasperetti, Alessio Autore, Camillo Casella, Michela Dello Russo, Antonio Stolfo, Davide Laredo, Mikael Gandjbakhch, Estelle Graziosi, Maddalena Biagini, Elena Catalano, Costantina Barile, Ludovica Drago, Fabrizio Cicenia, Marianna Baban, Anwar Pelargonio, Gemma Narducci, Maria Lucia Re, Federica Peretto, Giovanni Paiotti, Elena Díez López, Carles Olivotto, Iacopo Gaita, Fiorenzo Sinagra, Gianfranco Novelli, Giuseppe |
| author |
Calò, Leonardo |
| author_facet |
Calò, Leonardo Crescenzi, Cinzia Di Marco, Andrea Fanisio, Francesca Romeo, Fabiana Gargaro, Alessio Martino, Annamaria Cappelletto, Chiara Merlo, Marco Targetti, Mattia Toso, Elisabetta Toto, Federica Musumeci, Maria Beatrice Tini, Giacomo Ciabatti, Michele Stefanini, Matteo Canestrelli, Stefano Fedele, Elisa Lanzillo, Chiara Fusco, Armando Sangiuolo, Federica Carla Radesich, Cinzia Perotto, Maria Pieroni, Maurizio Mango, Ruggiero Gasperetti, Alessio Autore, Camillo Casella, Michela Dello Russo, Antonio Stolfo, Davide Laredo, Mikael Gandjbakhch, Estelle Graziosi, Maddalena Biagini, Elena Catalano, Costantina Barile, Ludovica Drago, Fabrizio Cicenia, Marianna Baban, Anwar Pelargonio, Gemma Narducci, Maria Lucia Re, Federica Peretto, Giovanni Paiotti, Elena Díez López, Carles Olivotto, Iacopo Gaita, Fiorenzo Sinagra, Gianfranco Novelli, Giuseppe |
| author_role |
author |
| author2 |
Crescenzi, Cinzia Di Marco, Andrea Fanisio, Francesca Romeo, Fabiana Gargaro, Alessio Martino, Annamaria Cappelletto, Chiara Merlo, Marco Targetti, Mattia Toso, Elisabetta Toto, Federica Musumeci, Maria Beatrice Tini, Giacomo Ciabatti, Michele Stefanini, Matteo Canestrelli, Stefano Fedele, Elisa Lanzillo, Chiara Fusco, Armando Sangiuolo, Federica Carla Radesich, Cinzia Perotto, Maria Pieroni, Maurizio Mango, Ruggiero Gasperetti, Alessio Autore, Camillo Casella, Michela Dello Russo, Antonio Stolfo, Davide Laredo, Mikael Gandjbakhch, Estelle Graziosi, Maddalena Biagini, Elena Catalano, Costantina Barile, Ludovica Drago, Fabrizio Cicenia, Marianna Baban, Anwar Pelargonio, Gemma Narducci, Maria Lucia Re, Federica Peretto, Giovanni Paiotti, Elena Díez López, Carles Olivotto, Iacopo Gaita, Fiorenzo Sinagra, Gianfranco Novelli, Giuseppe |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Miocardiopaties Desfibril·ladors cardioversors implantables Myocardiopathies Implantable cardioverter-defibrillators |
| topic |
Miocardiopaties Desfibril·ladors cardioversors implantables Myocardiopathies Implantable cardioverter-defibrillators |
| description |
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. (JACC Adv. 2025;4:101766) (c) 2025 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY license (http://creativecommons. org/licenses/by/4.0/). |
| publishDate |
2025 |
| dc.date.none.fl_str_mv |
2025 2025 2025 2025 |
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info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
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article |
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publishedVersion |
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https://hdl.handle.net/2445/222225 |
| url |
https://hdl.handle.net/2445/222225 |
| dc.language.none.fl_str_mv |
Inglés |
| language_invalid_str_mv |
Inglés |
| dc.relation.none.fl_str_mv |
Reproducció del document publicat a: https://doi.org/10.1016/j.jacadv.2025.101766 JACC Advances, 2025, vol. 4, num. 6 https://doi.org/10.1016/j.jacadv.2025.101766 |
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cc by (c) Calò, Leonardo et al, 2025 http://creativecommons.org/licenses/by/3.0/es/ info:eu-repo/semantics/openAccess |
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cc by (c) Calò, Leonardo et al, 2025 http://creativecommons.org/licenses/by/3.0/es/ |
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openAccess |
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15 p. application/pdf |
| dc.publisher.none.fl_str_mv |
Elsevier BV |
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Elsevier BV |
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Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) reponame:Recercat. Dipósit de la Recerca de Catalunya instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya) |
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Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya) |
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Recercat. Dipósit de la Recerca de Catalunya |
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Recercat. Dipósit de la Recerca de Catalunya |
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