Abstract 11877: Arrhythmogenic Right Ventricular Cardiomyopathy Patients with a Markedly Enlarged RV Compressing LV to Left Side Have an Atypical Distribution of Epsilon Waves and Elevated Plasma BNP

Abstract

Introduction: Epsilon waves on V1-3 leads are specific ECG findings in patients with arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC). 4D cardiac CT visualizes ARVC characteristics, such as fibro-fatty invasion into RV and left ventricular (LV) myocardium (RVM, LVM), an enlarged RV, reduced RV motion, and bulging. Hypothesis: We hypothesize that epsilon waves in V4-6 leads suggest LV invasion in ARVC. Alternatively, extreme RV enlargement may compress the LV and cause clockwise rotation; an enlarged RV may itself cause epsilon waves in V4-6 leads. Methods: Retrospective analysis of 17 patients (11 males, 57±17 yrs) with suspected ARVC undergoing cardiac CT and ECG, 9 of whom met 2010 ARVC task force criteria. Result: All 9 patients had epsilon waves ; 5 had fibro-fatty invasion into the LVM. We divided the 9 into 5 groups by CT: 1) markedly enlarged RV compressing the LV to the left side with fibro-fatty changes exclusively in RVM (N=1); 2) similar findings in both RVM and LVM (N=2); 3) moderately enlarged RV without compression of the LV to the left side and fibro-fatty changes exclusively in RVM (N=3); 4) the same in both RVM and LVM (N=2); 5) severe mitral valve regurgitation, a markedly enlarged LV, and a fibro-fatty change in both RVM and LVM (N=1). The patient in group (gp) 1 showed epsilon waves in V1-6 leads, patients in gp 2 had epsilon waves in V1-6 (N=1), and V3-5 (N=1) leads; patients in gp 3 had epsilon waves in V1-4 (N=2), and V1-3 (N=1) leads, patients in gp 4 had epsilon waves in V1-3 (N=1), and V1, 2 (N=1) leads; finally, the patient in gp 5 had epsilon waves in V4-6 leads. Plasma BNP levels were significantly greater in patients in gp 1 & 2 than gp 3 & 4 (1255±838 vs 80±52 pg/ml, P=0.016). Conclusions: ARVC patients with a markedly enlarged RV compressing the LV to the left side (gp 1,2) had a broad (V1-6) or different range (V3-5) distribution of epsilon waves and significantly elevated plasma BNP independent of fibro-fatty invasion of the LV, different from typical ARVC (gp 3,4).