Cardiomyopathy I42.80

Group of primary or secondary diseases of the heart muscle, which are accompanied by mechanical or electrical dysfunction and, depending on their severity, cause ventricular hypertrophy or dilatation.

The individual clinical pictures differ with regard to etiology, pathogenesis, clinical course and therapy. A classification is made either according to functional or aetiological criteria. Hybrid and transitional forms are observed, such as the phenotypes dilated cardiomyopathy, hypertrophic cardiomyopathy and restrictive cardiomyopathy within a family with the same mutation.

Classification according to anatomical and physiological characteristics:

• Hypertrophic cardiomyopathy (HCM)
• restrictive cardiomyopathy (RCM)
• Dilated cardiomyopathy (DCM)
• Arrhythmogenic right ventricular cardiomyopathy (ARVC)

Dilated cardiomyopathy is responsible for 60%, hypertrophic cardiomyopathy for about 30%, restrictive, arrythmogenic and unclassifiable cardiomyopathies for about 1-2% of the total cardiomyopathies.

WHO classification of specific (secondary) cardiomyopathies according to their etiology

• Inflammatory cardiomyopathy (autoimmune reaction, infectious or idiopathic chronic myocarditis)
• Ischaemic cardiomyopathy with coronary heart disease / heart attack
• Hypertensive cardiomyopathy in long-term hypertension
• Valvular cardiomyopathy due to chronic pressure and/or volume loading
• Metabolic cardiomyopathy (diabetes mellitus, hyper- or hypothyroidism, storage diseases, deficiency diseases, collagenosis, rheumatoid arthritis, vasculitis, etc.)
• Toxic cardiomyopathy (alcohol and toxic drugs)
• Peripartal (postpartum) cardiomyopathy
• Tachycardia cardiomyopathy (in tachycardic rhythm disorders)
• Unclassifiable cardiomyopathy

In recent years, genetic changes have been detected for many cardiomyopathies. These findings may lead to new classifications.

1. Battegay E (2017) Cardiomyopathies - Definition and classification. In: E.Battegay (Hrsg)Differential diagnosis of internal diseases. Georg Thieme publishing house. Stuttgart New York p 273
2. Salman OF et al (2018) Inherited Cardiomyopathies and the Role of Mutations in Non-coding Regions of the Genome.Front Cardiovasc Med 5:77.