RT Journal Article T1 Layer-Specific Global Longitudinal Strain Predicts Arrhythmic Risk in Arrhythmogenic Cardiomyopathy A1 Segura-Rodriguez, Diego A1 Bermudez-Jimenez, Francisco Jose A1 Gonzalez-Camacho, Lorena A1 Moreno Escobar, Eduardo A1 Garcia-Orta, Rocio A1 Alcala-Lopez, Juan Emilio A1 Bautista Paves, Alicia A1 Oyonarte-Ramirez, Jose Manuel A1 Lopez-Fernandez, Silvia A1 Alvarez, Miguel A1 Tercedor, Luis A1 Jimenez-Jaimez, Juan K1 sudden cardiac death (SCD) K1 late gadolinium enhanced K1 non-sustained ventricular tachycardia K1 arrhythmogenic cardiomyopathy (ACM) K1 global longitudinal strain K1 Right-ventricular cardiomyopathy/dysplasia K1 Echocardiographic reference ranges K1 Speckle-tracking echocardiography K1 Myocardial strain K1 Task-force K1 Diagnosis K1 Mutation K1 Association K1 Standard AB Background: Arrhythmogenic cardiomyopathy (AC) is a life-threatening disease which predispose to malignant arrhythmias and sudden cardiac death (SCD) in the early stages of the disease. Risk stratification relies on the electrical, genetic, and imaging data. Our study aimed to investigate how myocardial deformation parameters may identify the subjects at risk of known predictors of major ventricular arrhythmias.Methods: A cohort of 45 subjects with definite or borderline diagnosis of AC was characterized using the advanced transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) and divided into the groups according to the potential arrhythmic risk markers, such as non-sustained ventricular tachycardia (NSVT), late gadolinium enhancement (LGE), and genetic status. Layer-specific global longitudinal strain (GLS) by TTE 2D speckle tracking was compared in patients with and without these arrhythmic risk markers.Results: In this study, 23 (51.1%) patients were men with mean age of 43 +/- 16 years. Next-generation sequencing identified a potential pathogenic mutation in 39 (86.7%) patients. Thirty-nine patients presented LGE (73.3%), mostly located at the subepicardial-to-mesocardial layers. A layer-specific-GLS analysis showed worse GLS values at the epicardial and mesocardial layers in the subjects with NSVT and LGE. The epicardial GLS values of -15.4 and -16.1% were the best cut-off values for identifying the individuals with NSVT and LGE, respectively, regardless of left ventricular ejection fraction (LVEF).Conclusions: The layer-specific GLS assessment identified the subjects with high-risk arrhythmic features in AC, such as NSVT and LGE. An epicardial GLS may emerge as a potential instrument for detecting the subjects at risk of SCD in AC. PB Frontiers media sa SN 2297-055X YR 2021 FD 2021-11-15 LK https://hdl.handle.net/10668/26209 UL https://hdl.handle.net/10668/26209 LA en DS RISalud RD Apr 9, 2025