Abstract
Desmin-related myopathy (DRM) is known to cause different types of cardiomyopathy. Late gadolinium enhancement cardiovascular magnetic resonance (CMR) has been shown to identify fibrosis in ischemic and non-ischemic cardiomyopathies. We present a rare case of desmin-related hypertrophic cardiomyopathy, CMR revealed fibrosis in the lateral wall of the left ventricle. CMR is superior to conventional echocardiography for the detection of myocardial fibrosis in desmin-related cardiomyopathy, which may be useful to detect early cardiac involvement and predict the patient prognosis.
MeSH terms
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Adolescent
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Cardiomyopathy, Hypertrophic / genetics
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Cardiomyopathy, Hypertrophic / pathology*
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Cardiomyopathy, Hypertrophic / physiopathology
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Contrast Media
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DNA Mutational Analysis
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Desmin / genetics*
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Female
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Fibrosis
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Gadolinium DTPA
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Heart Ventricles / pathology
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Heart Ventricles / physiopathology
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Humans
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Magnetic Resonance Imaging*
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Myocardium / pathology*
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Myositis, Inclusion Body / genetics
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Myositis, Inclusion Body / pathology
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Myositis, Inclusion Body / physiopathology
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Predictive Value of Tests
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Sequence Deletion
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Ventricular Function, Left
Substances
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Contrast Media
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Desmin
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Gadolinium DTPA