Exercise: A Risky Subject in Arrhythmogenic Cardiomyopathy

Cerrone, Marina

doi:10.1161/jaha.118.009611

Cited by 7 publications

(6 citation statements)

References 14 publications

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“…The compromised heart of ACM patients undergoes a functional worsening when subject to intense physical exercise. The practice of physical activity at a competitive level represents one of the major triggers for life-threatening arrhythmias and sudden death in the ACM setting and therefore it is highly discouraged for ACM patients (Cerrone, 2018). Endurance athletes become symptomatic at an earlier age, more likely develop an overt phenotype, and show more frequently ventricular arrhythmias and heart failure (James et al, 2013).…”

Section: Pro-fibrotic Triggersmentioning

confidence: 99%

Fibrosis in Arrhythmogenic Cardiomyopathy: The Phantom Thread in the Fibro-Adipose Tissue

et al. 2020

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Arrhythmogenic cardiomyopathy (ACM) is an inherited heart disorder, predisposing to malignant ventricular arrhythmias leading to sudden cardiac death, particularly in young and athletic patients. Pathological features include a progressive loss of myocardium with fibrous or fibro-fatty substitution. During the last few decades, different clinical aspects of ACM have been well investigated but still little is known about the molecular mechanisms that underlie ACM pathogenesis, leading to these phenotypes. In about 50% of ACM patients, a genetic mutation, predominantly in genes that encode for desmosomal proteins, has been identified. However, the mutation-associated mechanisms, causing the observed cardiac phenotype are not always clear. Until now, the attention has been principally focused on the study of molecular mechanisms that lead to a prominent myocardium adipose substitution, an uncommon marker for a cardiac disease, thus often recognized as hallmark of ACM. Nonetheless, based on Task Force Criteria for the diagnosis of ACM, cardiomyocytes death associated with fibrous replacement of the ventricular free wall must be considered the main tissue feature in ACM patients. For this reason, it urges to investigate ACM cardiac fibrosis. In this review, we give an overview on the cellular effectors, possible triggers, and molecular mechanisms that could be responsible for the ventricular fibrotic remodeling in ACM patients.

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Section: Pro-fibrotic Triggersmentioning

confidence: 99%

Fibrosis in Arrhythmogenic Cardiomyopathy: The Phantom Thread in the Fibro-Adipose Tissue

et al. 2020

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“…A consensus correlation between exercise and increased risk of sudden cardiac death (SCD) restricts patients from physical activities. In truth, patients diagnosed with ACM usually have a complete change of lifestyle in order to prevent disease progression [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Arrhythmogenic Cardiomyopathy: Exercise Pitfalls, Role of Connexin-43, and Moving beyond Antiarrhythmics

Coscarella

Landim-Vieira

Pinto

et al. 2022

IJMS

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Arrhythmogenic Cardiomyopathy (ACM), a Mendelian disorder that can affect both left and right ventricles, is most often associated with pathogenic desmosomal variants that can lead to fibrofatty replacement of the myocardium, a pathological hallmark of this disease. Current therapies are aimed to prevent the worsening of disease phenotypes and sudden cardiac death (SCD). Despite the use of implantable cardioverter defibrillators (ICDs) there is no present therapy that would mitigate the loss in electrical signal and propagation by these fibrofatty barriers. Recent studies have shown the influence of forced vs. voluntary exercise in a variety of healthy and diseased mice; more specifically, that exercised mice show increased Connexin-43 (Cx43) expression levels. Fascinatingly, increased Cx43 expression ameliorated the abnormal electrical signal conduction in the myocardium of diseased mice. These findings point to a major translational pitfall in current therapeutics for ACM patients, who are advised to completely cease exercising and already demonstrate reduced Cx43 levels at the myocyte intercalated disc. Considering cardiac dysfunction in ACM arises from the loss of cardiomyocytes and electrical signal conduction abnormalities, an increase in Cx43 expression—promoted by low to moderate intensity exercise and/or gene therapy—could very well improve cardiac function in ACM patients.

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“…Exercise is one of the main triggers for life-threatening arrhythmias and SCD in several conditions that are vulnerable to adrenergic stress, in particular inherited diseases syndromes including AC (Cerrone, 2018). In the setting of AC, this can be mechanistically linked to: (i) a direct mechanical stress in cells with weakened cell-cell junctions, (ii) adrenergic surge, and (iii) electrophysiological remodeling.…”

Section: Resultsmentioning

confidence: 99%

Mechanotransduction and Adrenergic Stimulation in Arrhythmogenic Cardiomyopathy: An Overview of in vitro and in vivo Models

2020

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Exercise: A Risky Subject in Arrhythmogenic Cardiomyopathy

Cited by 7 publications

References 14 publications

Fibrosis in Arrhythmogenic Cardiomyopathy: The Phantom Thread in the Fibro-Adipose Tissue

Fibrosis in Arrhythmogenic Cardiomyopathy: The Phantom Thread in the Fibro-Adipose Tissue

Arrhythmogenic Cardiomyopathy: Exercise Pitfalls, Role of Connexin-43, and Moving beyond Antiarrhythmics

Mechanotransduction and Adrenergic Stimulation in Arrhythmogenic Cardiomyopathy: An Overview of in vitro and in vivo Models

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