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(5) Multimodality imaging to characterize the cardiac phenotype (morphology and function)—including tissue characterization for non-ischaemic myocardial scar detection—is necessary, in combination with a detailed personal and family history, clinical examination, electrocardiography, and laboratory investigations. (6) Imaging results should always be interpreted in the overall clinical context, including genetic testing results, rather than in isolation. (7) Tissue characterization by CMR is of value in diagnosis, monitoring of disease progression and risk stratification in each of the main cardiomyopathy phenotypes. (8) DPD/PYP/HMDP bone-tracer scintigraphy or SPECT represent the gold standard for the diagnosis of ATTR-related cardiac amyloidosis. (9) The presence of non-ischaemic ventricular scar or fatty replacement on cardiac CMR and/or pathological examination, which can occur with or without ventricular dilatation and/or systolic dysfunction, can be the sole clue to the diagnosis of a cardiomyopathy and can have prognostic significance that varies with aetiology. (10) The aim of this multiparametric and systemic approach is to generate a phenotype-based aetiological diagnosis, interpreting available data with a cardiomyopathy-oriented mindset that combines cardiological assessment with non-cardiac parameters. (11) A multidisciplinary approach to patient care and appropriate transition of care from paediatric to adult cardiomyopathy services is needed. (12) Genetic testing should be performed in patients with cardiomyopathy and may influence risk stratification and management. (13) Genetic counselling, including pre- and post-test counselling, and psychological support are an essential aspect of the multidisciplinary care of patients with cardiomyopathy and their relatives. (14) Paediatric cardiomyopathies largely represent part of the same clinical spectrum as those seen in older adolescents and adults, but infant-onset (in the first year of life) cardiomyopathies are often associated with severe phenotypes and a high rate of heart failure-related morbidity and mortality. (15) Beyond the first year of life, genetic causes of childhood-onset cardiomyopathies are similar to those in adults. (16) Symptom management, identification, and prevention of disease-related complications (including SCD, heart failure, and stroke) are the cornerstone of management of all cardiomyopathies. (17) Cardiac myosin inhibitors (Mavacamten) should be considered in patients with HCM and LVOTO who remain symptomatic despite optimal medical therapy. (18) Validated SCD risk-prediction tools (HCM Risk-SCD and HCM Risk-Kids) are the first step in sudden death prevention in patients with HCM. (19) Additional risk markers may be of use in patients with low or intermediate risk, but there is a lack of robust data on the impact of these parameters on the personalized risk estimates generated by the risk-prediction tools. (20) Pharmacological treatment of DCM patients does not differ from those recommended in chronic heart failure. (21) SCD risk of DCM and NDLVC patients varies depending on the underlying cause and genetic subtype. (22) CMR findings play an important role in guiding ICD implantation for patients with DCM and NDLVC. (23) In DCM and NDLVC patients, ICD should be considered for cer- tain genetic forms even if LVEF is >35%. (24) It is of importance to define aetiology for a tailored management in patients with syndromic and metabolic cardiomyopathies (i.e. ERT/chaperone in lysosomal storage disease; tafamidis in ATTRwt, etc.). (25) Pregnancy and the post-partum period are associated with increased cardiovascular risk in women with known cardiomyopathy. (26) A multidisciplinary team should evaluate the patient with cardiomyopathy to assess the risk associated with pregnancy. (27) Beta-blocker therapy on arrhythmic indication can safely be continued during pregnancy; safety data should be checked before initiation of new drugs in pregnancy. (28) Healthy adults of all ages and individuals with known cardiac disease should exercise with moderate intensity, totalling at least 150 min per week. (29) All patients with cardiomyopathy should have an individualized risk assessment for exercise prescription. Evaluation should be guided by three principles: (i) preventing life-threatening arrhythmias during exercise; (ii) symptom management to allow sports; and (iii) preventing sports-induced progression of the arrhythmogenic condition. (30) Individuals who are genotype positive/phenotype negative or have a mild cardiomyopathy phenotype and absence of symptoms or any risk factors, may be able to participate in competitive sports. In some high-risk patients with HCM, ARVC, and NDLVC, highintensity exercise and competitive sports should be discouraged. (31) Patients with high-risk genotypes or associated factors for arrhythmic or heart failure complications or severe LVOTO should be referred for specialized investigations before undergoing elective NCS. (32) Identification and management of risk factors and concomitant diseases is recommended as an integral part of the management of cardiomyopathy patients. 15 Gaps in evidence Although there have been major advances in the genetics, diagnosis, and treatments of patients with cardiomyopathy over the last few years, there are a number of areas where robust evidence is still lacking and deserve to be addressed in future clinical research. (1) Cardiomyopathy phenotypes. (2) Epidemiology: (a) Prevalence of NDLVC phenotype (children and adults). (b) Systematic assessment of prevalence of cardiomyopathy phenotypes in childhood. (3) Integrated patient management: (a) Embedding of telemedicine into cardiomyopathy networks. (4) Patient pathway: (a) Laboratory tests: (i) Studies on novel ‘omic’ biomarkers (proteomics, metabolomics, and transcriptomics) are needed to assess their potential value for diagnostic and prognostic purposes in cardiomyopathies. (b) Multimodality imaging: (i) Advanced echocardiographic techniques, including speckle tracking deformation imaging, are promising but lack robust validation in the setting of cardiomyopathies. ESC Guidelines 3589 Downloaded from academic.oup.com/eurheartj/article/44/37/3503/7246608 by guest on 07 October 2026