The inherited cardiomyopathies are primary disorders of the heart muscle with a genetic basis. On long-term follow-up they are associated with adverse outcomes, particularly sudden death, arrhythmia and heart failure.Load more
The inherited cardiomyopathies are primary disorders of the heart muscle with a genetic basis. On long-term follow-up they are associated with adverse outcomes, particularly sudden death, arrhythmia and heart failure.
The diagnosis is made on the basis of clinical, cardiac imaging (primarily with transthoracic echocardiography) and genetic features. Inherited cardiomyopathies are typically defined according to the findings on imaging findings than their genetic basis. There are a range of Inherited cardiomyopathies, including hypertrophic, dilated, right ventricular, restrictive and unclassified phenotypes. Due to the overlap in these phenotypes, the diagnosis can be challenging, and should ideally be done in a tertiary inherited cardiac conditions service that involves both cardiologists and clinical geneticists.
Familial restrictive cardiomyopathy is rare and is defined by a restrictive filling pattern on echocardiography.Load Less
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