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Evolving spectrum of arrhythmogenic cardiomyopathy: Implications for Sports Cardiology
Francesca Graziano1, Alberto Cipriani1, Dorottya Balla2
1Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Padova, Italy.
Insights
Arrhythmogenic cardiomyopathy (ACM) is a genetic heart condition causing arrhythmias. Early screening in athletes is crucial, especially for left-dominant variants, as exercise can trigger dangerous heart events.
Area of Science:
- Cardiology
- Genetics
- Sports Medicine
Background:
- Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease characterized by fibro-fatty myocardial replacement.
- The disease spectrum includes right ventricular, biventricular, and left dominant phenotypes.
- Athletic activity can exacerbate ACM, potentially leading to ventricular arrhythmias (VAs) and sudden cardiac death (SCD).
Purpose of the Study:
- To review the literature on ACM in the context of Sport Cardiology.
- To highlight the implications of ACM phenotypes in athletes.
- To emphasize diagnostic strategies for ACM variants in sports preparticipation screening (PPS).
Main Methods:
- Literature review focusing on ACM and its relationship with sports cardiology.
- Analysis of diagnostic challenges, particularly for left-dominant ACM variants.
- Evaluation of the role of exercise testing in athlete screening.
Main Results:
- Cardiac magnetic resonance imaging has expanded the understanding of ACM phenotypes.
- Left-dominant ACM variants may present with normal ECG and echocardiography, primarily manifesting as exercise-induced VAs.
- Exercise testing can improve the diagnostic yield of PPS for identifying ACM in athletes.
Conclusions:
- Early identification of ACM in athletes is vital for preventing adverse events.
- Preparticipation screening protocols should consider exercise testing to detect subtle ACM variants.
- While competitive sports are contraindicated, supervised low-to-moderate intensity physical activity may be permissible for ACM patients.
Abstract:
Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease, structurally characterized by progressive fibro-fatty replacement of the normal myocardium and clinically by ventricular arrhythmias (VAs). Predominantly thanks to the use of cardiac magnetic resonance, we have learnt that the spectrum of the disease encompasses not only the classical right ventricular phenotype, but also biventricular and left dominant variants. Sport activity contributes to the phenotypic expression and progression of ACM and may trigger life-threatening VAs and sudden cardiac death (SCD). We conducted a review of the literature about ACM and its implications in Sport Cardiology and summarized the main findings in this topic. Early identification of affected athletes through preparticipation screening (PPS) is fundamental but, while classical right-ventricular or biventricular phenotypes are usually suspected because of electrocardiogram (ECG) and echocardiographic abnormalities, variants with predominant left ventricular involvement are often characterized by normal ECG and unremarkable echocardiography. Usually the only manifestations of such variants are exercise-induced VAs and for this reason exercise testing may empower the diagnostic yield of the PPS. Patients with ACM are not eligible to competitive sports activity, but low-to-moderate intensity physical activity under medical supervision is possible in most cases.
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