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The role of MicroRNAs in arrhythmogenic right ventricular cardiomyopathy: A systematic review.
Kardiol Pol
January 2025
Department of Congenital Heart Diseases, Cardinal Stefan Wyszyński National Institute of Cardiology, Warszawa, Poland.
Endurance Exercise Promotes Episodes of Myocardial Injury in Individuals with a Pathogenic Desmoplakin (DSP) Variant.
Heart Rhythm
December 2024
Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA.
Background: Desmoplakin (DSP) variants are associated with left-predominant or biventricular arrhythmogenic cardiomyopathy. Exercise promotes penetrance and sustained ventricular arrhythmias (VA) in right-sided arrhythmogenic right ventricular cardiomyopathy, but its effect is unknown in DSP variant carriers.
Objectives: To assess whether exercise is associated with clinical outcomes among individuals with a pathogenic or likely pathogenic (P/LP) DSP variant.
Ventricular Discordance as an MRI Phenotype Provides Prognostic Value Among Arrhythmogenic Cardiomyopathy.
J Magn Reson Imaging
December 2024
Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Background: In arrhythmogenic cardiomyopathy (ACM), left ventricle-dominant presentation has poorer outcomes than right-dominant presentation, suggesting that interventricular functional disparity might play a role in patients' prognosis. However, the prognostic impact of ventricular functional discordance in ACM patients remains unknown.
Purpose: To assess whether ventricular functional disparity measured as ventricular discordance index, defined as the ratio of right-ventricular ejection fraction (RVEF) to left-ventricular ejection fraction (LVEF), might reveal prognostic disparities between phenotypes and offer added risk stratification value.
K currents in ventricular cardiomyocytes of p.N98S-calmodulin mutant mice.
Am J Physiol Heart Circ Physiol
December 2024
Wells Center for Pediatric Research, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.
Missense mutations in calmodulin (CaM)-encoding genes are associated with life-threatening ventricular arrhythmia syndromes. Here, we investigated a role of cardiac K channel dysregulation in arrhythmogenic long QT syndrome (LQTS) using a knock-in mouse model heterozygous for a recurrent mutation (p.N98S) in the gene (Calm1).
View Article and Find Full Text PDFA new prediction model for sustained ventricular tachycardia in arrhythmogenic cardiomyopathy.
Front Cardiovasc Med
December 2024
Department of Cardiology, Shanghai East Hospital, Tongji University School of Medicine, Shanghai, China.
Background: Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy characterized by high risks of sustained ventricular tachycardia (sVT) and sudden cardiac death. Identifying patients with high risk of sVT is crucial for the management of ACM.
Methods: A total of 147 ACM patients were retrospectively enrolled in the observational study and divided into training and validation groups.
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