Dyssynchrony Assessment in Arrhythmogenic Cardiomyopathy With Left Ventricular Involvement

Abstract

Arrhythmogenic cardiomyopathy (AC) is an inherited rare disease that can cause sudden cardiac death. Due to its heterogeneous phenotipe, a compendium of criteria collected in a Task Force Criteria (TFC) have to be met for its diagnosis, being dysshynchrony one of those criteria. The classical form of the disease involves the right ventricle, while the left ventricle AC (AC-LV) has been less studied. We aim to study dyssynchrony in AC-LV patients. The sample consisted of 36 subjects diagnosed with AC-LV and 23 non-affected relatives who were non-carriers of the pathogenic mutation of the proband. From each individual, radial, circumferential and longitudinal strain were obtained in the 16 AHA segments of the myocardium and dyssynchrony was calculated as the standard deviation of the time-to-peak strain. LV ejection fraction (LVEF) was also obtained. Finally, a clustering algorithm was applied to the 3-axis dyssynchronies and the LVEF. The clustering algorithm performed well (silhouette = 0.6) and detected 2 clusters (cluster1 = 23 controls + 19 AC-LV patients and cluster2 = 17 AC-LV patients). Radial dyssynchrony was the feature with the higher predictor importance. In conclusion, 47% of AC-LV patients present impaired dyssynchrony. Radial dyssynchrony was the most affected parameter among all dyssynchronies and LVEF.