Arrhythmogenic right ventricular cardiomyopathy

AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease primarily affecting the right ventricle and potentially causing sudden death in young people. Our aims are to analyse the progression over time of electrocardiographic (ECG) findings and to investigate their prognostic impact. METHODS: Sixty-eight patients (69% men; age 31 ± 19 years) with ARVC diagnosis were followed up for a mean of 17 ± 8 years. Follow-up included baseline ECG, 24-h Holter ECG, signal-averaged ECG, stress test, echocardiography, cardiac magnetic resonance and electrophysiologic study. RESULTS: During follow-up 12 (18%) patients died: three of sudden cardiac death (SCD), four of end-stage heart failure and five of noncardiac causes. Aborted SCD occurred in 7 (10%) patients, syncope in 31 (46%), sustained ventricular tachycardia in 43 (63%), heart failure in 18 (26%), atrial fibrillation in 16 (24%) and 3 (4%) patients underwent heart transplant. Twenty-four (35%) patients had implantable cardiac defibrillator (15 and 5 of them received both appropriate and inappropriate interventions, respectively and 7 experienced device-related complications). Of the ECG parameters registered at the enrolment, left anterior fascicular block (P = 0.001), QRS duration in lead 1 (P < 0.001), Epsilon wave (P < 0.001), T wave inversion in V4-V5-V6 (P = 0.012, P = 0.001 and P = 0.006) and low QRS voltages (P = 0.001) progressed over time. At multivariate analysis Epsilon wave (odds ratio 20.9, confidence interval 95% 1.8-239.8, P = 0.015) was the only predictor of the composite endpoint of SCD, heart failure-related death or heart transplant. CONCLUSION: Apart from playing a pivotal role in ARVC diagnosis, a simple ECG feature such as Epsilon wave is a marker of poor prognosis.