Noninvasive imaging is crucial for diagnosing and managing arrhythmogenic cardiomyopathy. Despite advanced multimodality imaging tools, challenges persist in differentiating it from other arrhythmogenic diseases (eg, cardiac sarcoidosis). We present a case of arrhythmogenic cardiomyopathy with an variant of uncertain significance exhibiting a phenocopy of cardiac sarcoidosis.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10874899 | PMC |
http://dx.doi.org/10.1016/j.jaccas.2023.102198 | DOI Listing |
Kardiol Pol
January 2025
Department of Congenital Heart Diseases, Cardinal Stefan Wyszyński National Institute of Cardiology, Warszawa, Poland.
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.
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.
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.
Heart Rhythm
December 2024
Cardiac Electrophysiology Section, Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio, USA. Electronic address:
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