Myocardial deformation techniques as potential tools to detect arrhythmogenic cardiomyopathy in early stages of the disease

Abstract Introduction Arrhythmogenic Cardiomyopathy (ACM) is a life-threatening entity which predispose to malignant arrhythmias and sudden cardiac death even in early stages of the disease. Deformation techniques obtained by echocardiography are promising tools which can identify subtle pathologic changes in the myocardial wall. Our aim is to investigate how myocardial deformation parameters may be affected throughout ACM spectrum. Methods A cohort of ACM 45 subjects, was characterized using advanced transthoracic echocardiography and divided into groups according to left ventricle ejection fraction (LVEF). Twenty-three healthy volunteers were also included as control group (CG). We analyzed regional wall motion abnormalities and left ventricular myocardial deformation parameters by 2D Speckle Tracking, such as global longitudinal strain (GLS), mechanical dispersion (MD) [standard deviation (SD) and range (delta)]. Results 23 (51,1%) of the ACM cohort were men, with a mean age of 43,13±16,55 years. Next-generation sequencing identified a potential pathogenic mutation in 37 (82,2%) of the patients. Those ACM subjects with low LVEF (ACM-L) presented lower GLS values when compared to those with normal LVEF (ACM-N) (−16,17±2,68% vs. ACM-N −19,39±2,97%; p