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.

JACC. Advances
|May 13, 2025
PubMed

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.
Abstract