Cardiomyopathy
Cardiomyopathy is a primary myocardial disorder classified by its structural and functional phenotype; it may present with heart failure, arrhythmia, syncope or a family history and requires specialist phenotyping, aetiological assessment, sudden-death risk review and family screening.
Definition
Cardiomyopathy is a myocardial disorder in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular disease or congenital heart disease sufficient to explain the abnormality. It is described by phenotype, including hypertrophic, dilated, restrictive, arrhythmogenic and non-dilated left-ventricular forms, and by its cause and disease phase.
First principles
Describe the phenotype before naming the cause
Cardiomyopathy is myocardial structural or functional abnormality not explained by coronary, hypertensive, valvular or congenital disease sufficient to account for it. Use a phenotype-first description: hypertrophic, dilated, restrictive, arrhythmogenic or non-dilated left-ventricular disease, then investigate genetic, inflammatory, infiltrative, toxic, metabolic, pregnancy-related and tachyarrhythmic causes. Phenotype, aetiology and phase of disease together determine management.
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