Abstract
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.
Highlights
Arrhythmogenic cardiomyopathy (AC) is a genetically determined myocardial disease characterized by the progressive fibro-fatty myocardial replacement leading to heart failure and life-threatening arrhythmias in the early stages of the disease [1]
This study aimed to evaluate the association of tissue imaging deformation (TID) with the major sudden cardiac death (SCD) risk factors as non-sustained ventricular arrhythmia, fibrous scar on cardiac magnetic resonance imaging (CMR), or genetic background
We thoroughly assessed the medical history for arrhythmic events: (a) non-sustained ventricular (NSVT) tachycardia defined as ≥3 consecutive premature complexes with a heart rate of >120 beats/min lasting 120 beats/min that lasts longer than 30 s, [2] the incidence aborted cardiac arrest due to VF which is reversed by the successful resuscitation maneuvers, and [3] the incidence of an appropriate implantable cardioverter-defibrillator (ICD) shock when they occurred in response to VT or VF
Summary
Arrhythmogenic cardiomyopathy (AC) is a genetically determined myocardial disease characterized by the progressive fibro-fatty myocardial replacement leading to heart failure and life-threatening arrhythmias in the early stages of the disease [1]. It is a clinically heterogeneous disease due to its incomplete penetrance and variable expression. The presence and location of late gadolinium enhancement (LGE) on CMR are reported as the predictive markers of ventricular arrhythmias and sudden cardiac death (SCD) [4, 5]. 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. Our study aimed to investigate how myocardial deformation parameters may identify the subjects at risk of known predictors of major ventricular arrhythmias
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