Correlation between signal-averaged ecg and the histologic evaluation of the myocardial substrate in right ventricular outflow tract arrhythmias

Background-The differential diagnosis between idiopathic and cardiomyopathy-related right ventricular outflow tract (RVOT) ventricular arrhythmias (VAs) is crucial. Signal-averaged ECG (SAECG) abnormalities are frequent in cardiomyopathy-related RVOT-VAs, although their pathophysiologic basis and diagnostic value in this setting are undefined. We tested the association between SAECG and the myocardial substrate underlying RVOT-VAs. Methods and Results-Twenty-four consecutive patients (median age, 50 years [42-59]; 12 men) with RVOT-VAs (10 with frequent [>1000/24 hours] premature ventricular contractions, 14 with ventricular tachycardias) underwent SAECG with 40-Hz filtering and electroanatomic mapping (EAM) with EAM-guided biopsy for characterization of the RVOT-VAs substrate. A filtered averaged QRS (fQRS) was obtained and analyzed for fQRS duration, low amplitude signal duration <40 mV (LAS40), and root-mean-square voltage in the last 40 ms of the QRS (RMS40). Standard definition of EAM scar was used. EAM-guided biopsy diagnosed ARVC in 11 (46%), myocarditis in 8 (33%), and idiopathic RVOT-VAs in 5 (21%) patients. Patients with cardiomyopathy-related RVOT-VAs had ≥1 EAM scar (median, 2 [1-2]; all with RVOT scar). EAM of patients with idiopathic RVOT-VAs was normal. Patients with cardiomyopathy-related RVOT-VAs had significantly longer fQRS (106 ms [92-132] versus 83 ms [82-84], P=0.01) and LAS40 (39 ms [36-51] versus 19 ms [18-21], P=0.02), and lower RMS40 (18 μV [9-26] versus 33 μV [32-33], P=0.04). A significant linear correlation was found between the extension (cm2) of the RVOT scar and all 3 SAECG parameters (r s=0.76, P<0.001 for the fQRSd; rs=0.73, P<0.001 for the LAS40; and rs=-0.72, P<0.001 for the RMS40). Using the established 2 of 3 criteria (ie, late potentials), SAECG diagnosed cardiomyopathy-related RVOT-VAs with high positive (100%) but low negative (38%) predictive values and missed 7 of 9 (78%) patients with RVOT scar <8 cm2. Conclusions-In patients with RVOT-VAs, abnormal SAECG parameters reflect the presence of extensive cardiomyopathic involvement of the RVOT. However, a negative SAECG does not reliably rule out cardiomyopathy-related RVOT-VAs in the presence of a small RVOT scar. (Circ Arrhythm Electrophysiol. 2012;5:475-483.)