Arrhythmogenic right ventricular cardiomyopathy, formerly called "arrhythmogenic right ventricular dysplasia," is an under-recognized clinical entity characterized by ventricular arrhythmias and a characteristic ventricular pathology. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore, consensus diagnostic criteria have been developed which combine electrocardiographic, echocardiographic, cardiac magnetic resonance imaging and histologic criteria. In 1994, an international task force first proposed the major and minor diagnostic criteria of arrhythmogenic right ventricular cardiomyopathy based on family history, arrhythmias, electrocardiographic abnormalities, tissue characterization, and structural and functional right ventricular abnormalities. In 2010, the task force criteria were revised to include quantitative abnormalities. These diagnostic modalities and the most recent task force criteria are discussed in this review.
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http://dx.doi.org/10.1097/CRD.0000000000000292 | 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.
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 PDFFront 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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