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5.5.2 Arrhythmogenic cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy (ARVC) is defined pathologically by the presence of fibro-fatty replacement of the right ventricle and clinically by life-threatening VAs. The condition was initially recognized as a predominantly RV disease, and diagnosis is currently based on probabilistic Task Force Criteria that encompass electrophysiological, anatomical, functional, and clinical features of the disease.375 Since its first description, the concept of ARVC has evolved to include concealed or subclinical phenotypes and biventricular disease. It is now well established that both ventricles are affected in most cases.376378 This has led to the development of a new term, arrhythmogenic cardiomyopathy, that embraces an array of diagnostic terms for different (genetic and acquired) pathologies. Although the definition of ‘arrhythmogenic cardiomyopathy’ is yet to be agreed, it can be considered as an umbrella term for a family of diseases that are characterized by biventricular myocardial abnormalities, including fibro-fatty infiltration and scarring, identified by pathological examination and/or cardiac imaging and VA.

The term arrhythmogenic cardiomyopathy (ACM) is used throughout these recommendations; however, it is important to recognize that most of the literature on the influence of exercise on disease progression and risk of SCD is derived from cohorts with classical ARVC. This is reflected in the recommendations provided in these Guidelines. It is possible therefore that the recommendations may not accurately reflect predominantly LV disease, which constitutes a small proportion of the disease spectrum where the impact of exercise on disease phenotype and risk is less clarified than the RV variant. Where appropriate, guidance is provided for other conditions that can be reasonably considered under the umbrella of ACM [including subtypes of dilated cardiomyopathy (DCM)].

5.5.2.1 Risk stratification in arrhythmogenic cardiomyopathy ACM accounts for a significant proportion of SCDs in young and athletic individuals.28 Established risk factors for SCD that should prompt consideration for an ICD include aborted SCD, unheralded syncope, ventricular tachycardia, and impaired RV and/or LV systolic function.379 A novel risk prediction model for VAs has recently been proposed but is yet to be validated.380 Regular and high-intensity exercise programmes are associated with acceleration of the disease process and worse outcomes.381389 In an experimental model of heterozygous plakoglobin-deficient mice, exercise training accelerated RV dysfunction and arrhythmias.382 Similar results have been confirmed in human desmosomal mutation carriers participating in vigorous (>70% VO2max) endurance sports.384 Similar findings were reported in patients with ACM and asymptomatic gene-positive family members, despite a more conservative definition of athletic status (exercise with intensity >_6 METs for >_4 h/week for >_6 years).386 Recently, the results from the North American multidisciplinary study reported that patients engaging in competitive sports were at two-fold increased risk of ventricular tachyarrhythmias or death and earlier presentation of symptoms, compared with patients who participated in recreational sports and sedentary individuals.385 Among patients engaging in competitive sports, early age of sports initiation was associated with premature presentation of symptoms and adverse clinical profile. Reducing exercise intensity was associated with a substantial decrease in the risk of ventricular tachyarrhythmias or death, to the same level as inactive patients.385 Finally, in a multinational registry of 393 competitive athletes implanted with an ICD who continued to participate in regular competitions, 20% of athletes with ACM received a shock during exertion compared to 10% at rest, during a median follow-up of 44 months. The diagnosis of ACM was the only variable associated with receiving appropriate shocks during competition.359,389

5.5.2.2 Baseline assessment of patients with arrhythmogenic cardiomyopathy A systematic approach is required when assessing individuals with ACM who request exercise advice. The baseline evaluation should include a comprehensive history of symptoms and family history of ACM or SCD, assessment of the severity of the ACM phenotype, and the presence of any conventional risk factors for SCD/SCA.

Recommendations for exercise and sports participation in individuals with hypertrophic cardiomyopathy

Recommendations Classa Levelb

Exercise recommendations Participation in high-intensity exercise/competitive sports, if desired (with the exception of those where occurrence of syncope may be associated with harm or death), may be considered for individuals who do not have any markers of increased riskc following expert assessment. IIb C Participation in low- or moderate-intensity recreational exercise, if desired, may be considered for individuals who have any markers of increased riskc following expert assessment . IIb C Participation in all competitive sports, if desired, may be considered for individuals who are gene positive for HCM but phenotype negative. IIb C Participation in high-intensity exercise (including recreational and competitive sports) is not recommended for individuals who have ANY markers of increased riskc. III C Follow-up and further considerations relating to risk Annual follow-up is recommended for individuals who exercise on a regular basis. I C Six-monthly follow-up should be considered in adolescent individuals and young adults who are more vulnerable to exercise-related SCD. IIa C Annual assessment should be considered for genotype-positive/phenotype-negative individuals for phenotypic features and risk stratification purposes. IIa C

BP = blood pressure; ESC = European Society of Cardiology; HCM = hypertrophic cardiomyopathy; LVOT = left ventricular outflow tract obstruction cardiomyopathy; SCD = sudden cardiac death. aClass of recommendation. bLevel of evidence. cMarkers of increased risk include: (i) cardiac symptoms or history of cardiac arrest or unexplained syncope; (ii) moderate ESC risk score (>_4%) at 5 years; (iii) LVOT gradient at rest >30 mmHg; (iv) abnormal BP response to exercise; (v) exercise-induced arrhythmias. Refer to Table 4 for different indices of exercise intensity and training zones. Downloaded from academic.oup.com/eurheartj/article/42/1/17/5898937 by guest on 07 October 2026