Arrhythmogenic right ventricular cardiomyopathy: ECG progression over time and correlation with long-term follow-up

Area of Science:

• Cardiology
• Genetics
• Electrophysiology

Background:

• Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac condition primarily affecting the right ventricle.
• It poses a significant risk of sudden cardiac death, particularly in younger individuals.
• Understanding the long-term progression of electrocardiographic (ECG) findings in ARVC is crucial for risk stratification.

Purpose of the Study:

• To analyze the temporal progression of ECG abnormalities in patients diagnosed with ARVC.
• To investigate the prognostic value of these ECG findings, particularly in relation to adverse cardiovascular events.

Main Methods:

• A cohort of 68 ARVC patients (69% male, mean age 31 years) was followed for a mean of 17 years.
• Comprehensive follow-up included baseline ECG, 24-h Holter monitoring, signal-averaged ECG, stress testing, echocardiography, cardiac MRI, and electrophysiologic studies.
• Statistical analysis, including multivariate analysis, was performed to identify predictors of adverse outcomes.

Main Results:

• During follow-up, 18% of patients died (SCD, heart failure, or non-cardiac causes), 10% experienced aborted SCD, and significant rates of ventricular tachycardia (63%) and syncope (46%) were observed.
• Progressive ECG changes included left anterior fascicular block, increased QRS duration, Epsilon waves, T-wave inversion in anterior leads, and low QRS voltages.
• Multivariate analysis identified the Epsilon wave as the sole significant predictor (OR 20.9) of a composite endpoint including SCD, heart failure-related death, or heart transplant.

Conclusions:

• The Epsilon wave is not only vital for ARVC diagnosis but also serves as a strong independent marker of poor prognosis.
• These findings underscore the importance of ECG monitoring in ARVC management and risk assessment.