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Inheritable cardiomyopathies 

Inheritable cardiomyopathies
Chapter:
Inheritable cardiomyopathies
DOI:
10.1093/med/9780199549573.003.07
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date: 18 October 2019

Hypertrophic cardiomyopathy 178

Dilated cardiomyopathy 182

Arrhythmogenic right ventricular cardiomyopathy 184

Left ventricular non-compaction 188

Anderson-Fabry disease 190

The commonest inheritable cardiomyopathy (prevalence 1 in 500), often with autosomal dominant inheritance. Typically asymmetric hypertrophy, particularly in the basal septum, which may lead to outflow tract obstruction (about 25% of patients). Can present with predominantly apical thickening [apical hypertrophic cardiomyopathy (HCM)] and concentric hypertrophy is also possible. Progressive LV dilatation can occur late in the disease process....

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