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................................................................ 5.5.2.3 History Syncope due to presumed arrhythmia is an important risk marker for SCD/SCA and a predictor of future appropriate ICD therapies.390394 The presence of symptoms attributed to ACM should reinforce the conservative exercise recommendations. Individuals with a history of cardiac arrest or unheralded syncope and individuals with exercise-induced symptoms should be advised to engage only in low-intensity recreational exercise programmes.

5.5.2.4 Resting and ambulatory ECG Apart from its diagnostic utility, the 12-lead ECG may provide useful information relating to risk stratification in ACM. The presence of extensive T-wave inversion affecting >_3 precordial leads or T-wave inversion in two of the three inferior leads confers some additional risk for SCD/ SCA.395,396 Ambulatory ECG monitoring is important for detecting VAs. Every effort should be made for the monitoring period to include the proposed exercise session. The presence of NSVT or significant burden of ventricular ectopy (>_1000/24 h), even in asymptomatic individuals, confers an increased risk of fatal arrhythmias.392,393,397

5.5.2.5 Echocardiography and cardiac magnetic resonance imaging In relation to risk stratification for SCD, the clinician should assess the severity of RV and LV involvement in terms of ventricular dilatation and systolic dysfunction. CMR imaging is more useful than echocardiography for assessing RV wall motion abnormalities and can also quantify the degree of myocardial fat infiltration and/or scar. The more extensive the disease the higher the arrhythmic risk.398,399

5.5.2.6 Exercise testing Exercise testing should be part of the routine assessment of every individual with ACM who wishes to exercise, as it can provide information regarding functional capacity and risk stratification. Exercise testing in patients with ACM should not be performed during ‘hot phases’. The presence of exercise-induced symptoms or arrhythmias should result in more conservative recommendations.

5.5.2.7 Genetic testing Genotype may also be of prognostic value. In the ARVC variant, a number of studies have reported that carriers of multiple pathogenic variants in the same desmosomal gene or mutations in >_2 genes may have an almost four-fold higher arrhythmic risk than those with a single mutation.400 Particular genotypes such as DSP and TMEM43, but also LMNA and FLNC, associated with other ACM phenotypes (see section 5.5.4) have a propensity for high arrhythmic burden that can pre-date the structural phenotype.401,402

5.5.2.8 Exercise recommendations The overall scientific evidence supports the concept that in patients with ACM participation in high-intensity sports should be discouraged, because it is associated with accelerated disease progression, greater risk of VAs and major events. This recommendation is also applicable to genetic carriers of pathogenic variants for ACM even in the absence of overt disease phenotype. 5.5.2.9 Special considerations Young age of presentation and male sex are associated with increased risk of malignant arrhythmias in ACM.379 Although young age should not exclude an individual from moderate-intensity exercise in the absence of high-risk features, age should be considered in the discussion with the patient and the parents. In addition, one should consider that specific highly dynamic, startstop sports, such as basketball and football, may pose a higher risk of SCD particularly in athletes who compete at the highest level.17,365

5.5.2.10 Follow-up An annual follow-up is recommended for most individuals with ACM who exercise on a regular basis. More frequent (6monthly) follow-up should be considered for adolescent and young adults whose ACM phenotype, and therefore risk of SCD, may still be evolving, particularly if they engage in moderate- to high-intensity exercise. More frequent follow-up should also be considered in individuals with high arrhythmic risk genotypes such as DSP, TMEM43, and carriers of multiple pathogenic variants. New symptoms should prompt interruption of exercise and reevaluation.

Recommendations for exercise and sports participation in individuals with arrhythmogenic cardiomyopathy

Recommendations Classa Levelb

Exercise recommendations Participation in 150 min of low-intensity exercise per week should be considered for all individuals. IIa C Participation in low- to moderate-intensity recreational exercise/sports, if desired, may be considered for individuals with no history of cardiac arrest/VA, unexplained syncope, minimal structural cardiac abnormalities, <500 PVCs/24 h and no evidence of exercise-induced complex VAs. IIb C Participation in high-intensity recreational exercise/ sports or any competitive sports is not recommended in individuals with ACM, including those who are gene positive but phenotype negative.384,386 III B 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 Six-monthly follow-up should also be considered in individuals with high arrhythmic risk genotypes such as DSP, TMEM43, and carriers of multiple pathogenic variants. IIa C

ACM = arrhythmogenic cardiomyopathy; PVC = premature ventricular contraction; SCD = sudden cardiac death; VA = ventricular arrhythmia. aClass of recommendation. bLevel of evidence. 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