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and have a lower risk of cerebrovascular accidents1085 and certain malignancies.1085–1087 These benefits that can be derived later in life also apply to individuals with established cardiovascular disease. For a definition of exercise intensity levels, please refer to Supplementary data online, Table S6.

8.1.2 Exercise-related sudden cardiac death and

historical exercise recommendations for patients with cardiomyopathy Rigorous exercise may trigger myocardial infarction and fatal arrhythmias among individuals with an underlying cardiovascular disease.1088– 1091 Superimposed on the pathological substrate of the disease entity itself, exercise may induce sudden cardiac arrest through mechanical shearing forces within the coronary arteries, effects of high concentrations of circulating catecholamines, increased cardiac loading conditions, raised core temperature, electrolyte shifts, and acid-base disturbance. Cardiomyopathies are the leading cause of exercise-related SCD in young people in the Western world.40,1092–1095 The established link between exercise and SCD from cardiomyopathy, and the finding that, in some cardiomyopathy phenotypes, exercise may accelerate progression of the underlying cardiomyopathic disease process, has historically resulted in restrictive exercise recommendations in all affected patients regardless of pathology, disease severity, symptomatic status, general risk profile, or prior therapeutic interventions, including an ICD.1096– 1098 As a result, individuals with cardiomyopathy often confine themselves to a relatively sedentary lifestyle through fear of potential SCD and accrue risk factors for atherosclerotic CAD, which confer a worse prognosis.1099–1102,1096,1097

8.1.3 Exercise recommendations in hypertrophic

cardiomyopathy Recent pre-clinical1103 and clinical data suggest that moderate exercise may be beneficial and safe in patients with HCM.1098–1102 Information on a safe dose of vigorous exercise is still limited, but the heterogeneous morphology and pathophysiology of HCM means that some individuals are capable of participating in vigorous exercise, including high-intensity competitive sports.760 Most athletes capable of exercising intensively have mild LV hypertrophy, normal-sized or enlarged LV, normal diastolic function, and no evidence of LVOTO.1104,1105 Currently available data indicate that participation in vigorous exercise and competitive sport may be considered in a select group of predominantly adult patients who have mild morphology and a low-risk profile.1106–1108 However, studies examining the effect of vigorous exercise or moderate-to-high-intensity competitive sport on the natural history of HCM were not designed or powered adequately to address the question and there are potential issues of selection bias. Nevertheless, based on emerging evidence, the Task Force agreed to adopt a comparatively liberal approach, advocating that, after appropriate selection, some individuals with a low-risk profile may participate in high-intensity exercise and competitive sport after comprehensive expert evaluation and shared discussion, which highlights the unpredictable nature of exercise-related SCD in HCM. Sporting disciplines in which syncope may result in fatal accidental injury or danger to others are not recommended. Genotype-positive/phenotype-negative patients may engage in all competitive sport; however, annual assessment is recommended to check for developing phenotypic features of disease.1109 8.1.4 Exercise recommendations in arrhythmogenic

right ventricular cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy is a recognized cause of exercise-related SCD in young asymptomatic individuals,40,890 postulated to result from ventricular stretch leading to myocyte detachment with subsequent inflammation and fibro-fatty replacement of the ventricular myocardium. Fatal arrhythmias may occur during the inflammatory process or because of myocardial scar. In addition, there are data to suggest that high-intensity exercise is associated with acceleration of disease phenotype in individuals with ARVC, including those who are genotype positive/phenotype negative, and particularly those with PKP2 variants.181,1110–1114 Furthermore, exercise restriction has been shown to improve clinical outcomes in patients with ARVC.40,1111,1115–1117 Based on these data, the Task Force recommends against intensive exercise or competitive sports in individuals with ARVC as part of a shared decision-making process. The evidence on the impact of exercise in genotype-positive/phenotype-negative individuals is more limited. In these cases, the Task Force recommends a cautious approach in the context of shared decision-making when discussing competitive sports participation. Mild-to-moderate physical activity for up to 150 min per week is considered safe and is recommended in able phenotype-negative individuals.1118

8.1.5 Exercise recommendations in dilated

cardiomyopathy and non-dilated left ventricular cardiomyopathy There is evidence that moderate exercise in optimally treated patients with DCM improves functional capacity, ventricular function, and QoL;1119 however, intensive exercise and competitive sports may also trigger fatal arrhythmias in DCM and NDLVC.1093,1120–1122 In general, symptomatic individuals with DCM and NDLVC should abstain from most competitive and leisure sports, or recreational exercise associated with moderate or high exercise intensity. A select group of asymptomatic individuals with DCM and NDLVC who have mildly impaired LV function without exercise-induced arrhythmias or significant myocardial fibrosis may participate in most competitive sports. Although the natural history of most pathogenic variants capable of causing DCM and NDLVC is unknown, it would be reasonable to permit intensive exercise and competitive sports in most individuals with pathogenic variants in the absence of overt features of DCM or NDLVC. Special consideration, however, should be given to individuals with pathogenic variants in genes that are associated with an increased risk of life-threatening arrhythmias, such as lamin A/C181,1123 or TMEM43 variants, for which there is emerging evidence that exercise may have an adverse effect on cardiac function and risk of potentially fatal arrhythmias. The impact of vigorous exercise in patients with pathogenic variants in other high-risk genes, such as filamin C variants1112 exhibiting DCM or NDLVC phenotypes, is not fully understood; however, extrapolating our understanding of the effect of exercise on some ARVC and DCM phenotypes necessitates a cautious approach.

Recommendation Table 31 — Exercise recommendations for patients with cardiomyopathy

Recommendations Classa Levelb

All cardiomyopathies Regular low- to moderate-intensity exercise is recommended in all able individuals with cardiomyopathy. I C

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