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The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy
Leonardo Calò1, Cinzia Crescenzi2, Andrea Di Marco3
1Division of Cardiology, Policlinico Casilino, Rome, Italy; Department of Motor, Human and Health Sciences, "Foro Italico" University of Rome, Rome, Italy.
Insights
Electrocardiogram (ECG) findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) are distinct and can predict major arrhythmic events. Left posterior fascicular block (LPFB) on ECG is a strong predictor of adverse outcomes in ALVC patients.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Electrocardiographic (ECG) findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) are not well-established.
- Previous studies on ALVC ECG characteristics were limited in scope.
Purpose of the Study:
- To analyze ECG characteristics in ALVC.
- To correlate ECG findings with cardiac magnetic resonance (CMR) and genetic data.
- To assess the prognostic value of ECG in ALVC.
Main Methods:
- Retrospective review of 125 ALVC patients, predominantly with desmoplakin variants.
- Analysis of electrocardiographic (ECG) patterns.
- Correlation with cardiac magnetic resonance (CMR) and genetic data.
- Evaluation of major arrhythmic events (MAEs) and their predictors using logistic and Cox regression.
Main Results:
- Distinct ECG signs identified in ALVC, including left posterior fascicular block (LPFB), pathological Q waves, and specific criteria for R/S ratios and voltage.
- Fifteen percent of patients had a normal ECG.
- Major arrhythmic events (MAEs) occurred in 28% of patients.
- Independent predictors of MAE included LPFB, syncope, and transmural late gadolinium enhancement on CMR.
- A predictive model incorporating these factors showed high accuracy (AUC=0.9).
Conclusions:
- ECG is a valuable diagnostic tool in ALVC.
- ECG possesses significant prognostic value in ALVC.
- Left posterior fascicular block (LPFB) is a strong, independent predictor of major arrhythmic events in ALVC.
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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