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(iv) differential disease expression in families; and (v) emerging aetiology-focused therapies. The Task Force concluded that a single classification system that embraces all possible causes of disease and every clinical scenario remains an aspiration that is outside the scope of this clinical guideline. Instead, the Task Force updated the existing clinical classification to include new phenotypic descriptions and to simplify terminology, while simultaneously providing a conceptual framework for diagnosis and treatment. This nomenclature prompts clinicians to consider cardiomyopathy as the cause of several clinical presentations (e.g. arrhythmia, heart failure), and focuses on morphological and functional characteristics of the myocardium (Figure 2). It is important to recognize that different

Suspected cardiomyopathy

Ventricular morphology/function Hypertrophy Dilatation Systolic/diastolic function Ventricular scar/fatty replacement

Non-ischaemic ventricular scar on CMR/pathological examination Other tissue characterization parameters on CMR

Symptoms Incidental findings Family screening Dyspnoea Chest pain Palpitation Syncope/presyncope Cardiac arrest 1st degree relative with CMP Family history of sudden death

Abnormal ECG Murmur Arrhythmia

Clinical scenario

Arrhythmias/conduction disease (atrial, ventricular, atrioventricular block)

Pedigree analysis

Laboratory markers Pathology

Genetic testing Extracardiac involvement

Phenotype-based integrated aetiological diagnosis

Phenotype HCM DCM ARVC

RCM NDLVC

Morphological/

functional characterization

Additional traits

Figure 2 Clinical diagnostic workflow of cardiomyopathy. ARVC, arrhythmogenic right ventricular cardiomyopathy; CMP, cardiomyopathy; CMR, cardiac magnetic resonance; DCM, dilated cardiomyopathy; ECG, electrocardiogram; HCM, hypertrophic cardiomyopathy; NDLVC, non-dilated left ventricular cardiomyopathy; RCM, restrictive cardiomyopathy.

ESC Guidelines                                                                                                                                                                                          3513

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