{"title":"12导联心电图对致心律失常左室心肌病的诊断及预后价值","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 , Giuseppe Novelli MD, PhD","doi":"10.1016/j.jacadv.2025.101766","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div>Electrocardiographic findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) have been limited to small studies.</div></div><div><h3>Objectives</h3><div>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.</div></div><div><h3>Methods</h3><div>We reviewed data of 125 consecutive patients with ALVC (81.5% <em>desmoplakin</em> 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.</div></div><div><h3>Results</h3><div>ALVC showed distinct ECG signs, including left posterior fascicular block (LPFB) (13.6%), pathological Q waves (26.4%), R/S ratio in V<sub>1</sub> ≥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.</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":73527,"journal":{"name":"JACC advances","volume":"4 6","pages":"Article 101766"},"PeriodicalIF":0.0000,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy\",\"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 , Giuseppe Novelli MD, PhD\",\"doi\":\"10.1016/j.jacadv.2025.101766\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Background</h3><div>Electrocardiographic findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) have been limited to small studies.</div></div><div><h3>Objectives</h3><div>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.</div></div><div><h3>Methods</h3><div>We reviewed data of 125 consecutive patients with ALVC (81.5% <em>desmoplakin</em> 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.</div></div><div><h3>Results</h3><div>ALVC showed distinct ECG signs, including left posterior fascicular block (LPFB) (13.6%), pathological Q waves (26.4%), R/S ratio in V<sub>1</sub> ≥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.</div></div><div><h3>Conclusions</h3><div>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.</div></div>\",\"PeriodicalId\":73527,\"journal\":{\"name\":\"JACC advances\",\"volume\":\"4 6\",\"pages\":\"Article 101766\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-05-12\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"JACC advances\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2772963X25001838\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"JACC advances","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2772963X25001838","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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 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.
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