Segura-Rodríguez, DiegoBermúdez-Jiménez, Francisco JoséGonzález-Camacho, LorenaMoreno Escobar, EduardoGarcía-Orta, RocíoAlcalá-López, Juan EmilioBautista Pavés, AliciaOyonarte-Ramírez, José ManuelLópez-Fernández, SilviaÁlvarez, MiguelTercedor, LuisJiménez-Jáimez, Juan2022-03-232022-03-232021-11-15Segura-Rodríguez D, Bermúdez-Jiménez FJ, González-Camacho L, Moreno Escobar E, García-Orta R, Alcalá-López JE, et al. Layer-Specific Global Longitudinal Strain Predicts Arrhythmic Risk in Arrhythmogenic Cardiomyopathy. Front Cardiovasc Med. 2021 Nov 15;8:748003.http://hdl.handle.net/10668/3488Background: 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.enAtribución 4.0 Internacionalhttp://creativecommons.org/licenses/by/4.0/Sudden cardiac death (SCD)Late gadolinium enhancedNon-sustained ventricular tachycardiaArrhythmogenic cardiomyopathy (ACM)Global longitudinal strainMuerte súbita cardíacaCardiopatíasTaquicardia ventricularMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Techniques, Cardiovascular::Heart Function Tests::Cardiac Output::Stroke VolumeMedical Subject Headings::Chemicals and Drugs::Inorganic Chemicals::Elements::Metals, Rare Earth::Lanthanoid Series Elements::GadoliniumMedical Subject Headings::Chemicals and Drugs::Chemical Actions and Uses::Pharmacologic Actions::Diagnostic Uses of Chemicals::Contrast MediaMedical Subject Headings::Diseases::Cardiovascular Diseases::Heart Diseases::Arrhythmias, Cardiac::Tachycardia::Tachycardia, VentricularMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Imaging::Cardiac Imaging Techniques::EchocardiographyMedical Subject Headings::Diseases::Cardiovascular Diseases::Heart Diseases::Arrhythmias, CardiacMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Genetic Techniques::Sequence Analysis::High-Throughput Nucleotide SequencingMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Chemistry Techniques, Analytical::Spectrum Analysis::Magnetic Resonance SpectroscopyMedical Subject Headings::Phenomena and Processes::Genetic Phenomena::Genetic Variation::Mutationresearch article34869653open access10.3389/fcvm.2021.7480032297-055XPMC8634435