The diagnosis of an inherited cardiomyopathy may lead to exclusion from competitive sports and affect decisions about having children. Some patients must live with a small risk of sudden death or the possibility of a future heart transplantation. An implantable cardioverter defibrillator (ICD) may be advised to prevent sudden death. The Guidelines recommend genetic counselling to support patients and their relatives understand and adapt to the diagnosis of a genetic disease. It should be offered before a decision to test is made and when the results are returned to the patient and their family. In addition, clinical psychological support should be offered to all patients who have undergone ICD implantation or who have a family history of sudden cardiac death.

For all cardiomyopathies, management aims to identify and treat symptoms, prevent complications and screen at-risk relatives. Identifying those at increased risk of sudden death is a major focus of these Guidelines. Shared decision-making is advised when selecting certain treatments such as ICDs. Other treatments include drugs to address symptoms and heart transplantation. Many subtypes are inherited in an autosomal dominant way, meaning that offspring have a 50% chance of inheriting the mutation. When such a mutation is found, testing of relatives can be considered. Dr. Kaski said: “Early diagnosis and treatment of family members can prevent disease progression in some cardiomyopathies and avoid complications.”


A dedicated section of the Guidelines provides advice for patients on living with a cardiomyopathy. This includes exercise, diet, alcohol, weight, reproductive issues, sexual activity, medication, vaccination, driving, employment, holidays and travel insurance, and life insurance.

Cardiomyopathies are the leading cause of exercise-related sudden cardiac death in young people in the Western world. In some cardiomyopathies, exercise accelerates progression of the disease, and this has historically resulted in recommendations for all patients to limit activity. Many patients have confined themselves to a relatively sedentary lifestyle through fear of sudden cardiac death and have missed out on the benefits of exercise in preventing coronary artery disease. These Guidelines recommend regular low to moderate intensity exercise for all patients with cardiomyopathy who are able to do so. An individualised risk assessment should be performed so that an exercise prescription can be provided taking into account the prevention of life-threatening arrhythmias during exercise, symptom management to allow sports and preventing sports-induced progression of the disease. Patients with certain cardiomyopathies should avoid very high intensity or competitive sports.

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