Characterization of the Left-Sided Substrate in Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

Background-The correlates of left ventricular (LV) substrate in arrhythmogenic right ventricular (RV) cardiomyopathy are largely unknown. Methods and Results-Thirty-two patients with arrhythmogenic RV cardiomyopathy (47±14 years; 6 women) were included. RV and LV dysplasia were defined from multidetector computed tomography and cardiac magnetic resonance imaging. Arrhythmias were characterized as right-sided or left-sided on 12-lead ECG recordings at baseline and during isoproterenol testing. In 14 patients, the imaging substrate was compared with voltage mapping and local abnormal ventricular activity. Imaging abnormalities were found in 32 (100%) and 21 (66%) patients on the RV and LV, respectively, intramyocardial fat on multidetector computed tomography being the most sensitive feature. LV involvement related to none of the Task Force criteria. Right-sided arrhythmias were more frequent than left-sided arrhythmias (P=0.003) although the latter were more frequent in case of LV involvement (P=0.02). The agreement between low voltage and fat on multidetector computed tomography was high on the RV when using either endocardial unipolar or epicardial bipolar data (κ=0.82 and κ=0.78, respectively) but lower on the LV (κ=0.54 for epicardial bipolar). LV local abnormal ventricular activity was found in all patients with LV involvement, and none of the others. The density of local abnormal ventricular activity within fat areas was similar between the RV and LV (P=0.57). Conclusions-LV substrate is frequent in arrhythmogenic RV cardiomyopathy, but poorly identified by current diagnostic strategies. Left-sided arrhythmias are more frequent in case of LV involvement. LV fat hosts the same density of local abnormal ventricular activity as RV fat, but is less efficiently detected by voltage mapping. These results support the need for alternative diagnostic strategies to identify LV dysplasia. (Circ Arrhythm Electrophysiol.

Multichannel electrocardiogram diagnostics for the diagnosis of arrhythmogenic right ventricular dysplasia

“…As its name suggests, it was initially felt to be a disease of the right ventricle but there is now evidence of left ventricular and bi-ventricular forms [9][10][11] . Those affected are often asymptomatic and the first manifestation can be with a sudden, unexplained death.…”

“…Based on this formal representation of ECG data as -dimensional vectors, we can define a distance function quantifying the dissimilarity between two time series and . A straightforward and commonly used measure is the Euclidean distance [8], however, in our analysis the dynamic time warping (DTW) distance was applied [9], in order to be able to address the problem of variation in the speed period of time series/heart rates between recordings from subjects..…”

Selecting the Appropriate Ablation Strategy: the Role of Endocardial and/or Epicardial Access

“…12 Involvement of the left ventricle is not uncommon, and is frequently subepicardial or intramural. 13 Often, to render patients with ARVC non-inducible, both epicardial and endocardial ablation procedures are necessary.…”

Arrhythmogenic cardiomyopathies (ACs): diagnosis, risk stratification and management

“…They most commonly arise from the RV in typical ARVC (figure 3), but left ventricular arrhythmias are seen in the biventricular and left-dominant subphenotypes 46…”