Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy, accounting for 90% of cases. Most common cause of heart transplants.
Causes:
- idiopathic: the most common cause
- myocarditis: e.g. Coxsackie B, Parvovirus B19, HSV, HIV, diphtheria, Chagas disease
- ischaemic heart disease
- peripartum
- hypertension
- iatrogenic: e.g. radiation, doxorubicin (adriamycin)
- substance abuse: e.g. alcohol, cocaine
- Toxins (cobalt, lead, arsenic)
- inherited: either a familial genetic predisposition to DCM or a specific syndrome e.g. Duchenne muscular dystrophy
- around a third of patients with DCM are thought to have a genetic predisposition
- a large number of heterogeneous defects have been identified
- the majority of defects are inherited in an autosomal dominant fashion although other patterns of inheritance are seen
- infiltrative e.g. haemochromatosis, sarcoidosis
- these causes may also lead to restrictive cardiomyopathy
- nutritional e.g. wet beriberi (thiamine deficiency)
- Metabolic: chronic hypophosphatemia, hypokalemia, hypocalcemia, uremia
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"ABCDE"
Alcohol, Beri beri, Chagas/Cocaine/Cockskie/Cyclophosphamide, Doxorubicin, Electrolyte disturbance
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Pathophysiology
- associated with mutations affecting cardiac cell cytoskeleton proteins and mitochondrial enzymes of oxidative phosphorylation in inherited forms which occupies about 35% of cases
- E.g, X-linked DCM involving the gene for dystrophin, which normally links the internal myocyte cytoskeleton with the external basement membrane. (similar process to Duchenne and Becker skeletal muscle dystrophies)
- dilated heart leading to predominately systolic dysfunction
- all 4 chambers are dilated, but the left ventricle more so than right ventricle