Pathology of Cardiomyopathy

Pathology
path of cardiomyopathy 5/22

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Text Dilated CardiomyopathyDefined by {{c1::progressive}} cardiac {{c1::dilatation}} and {{c1::contractile}} dysfunction usually with concomitant {{c1::hypertrophy}} Characterized by: -{{c1::Global}} {{c1::left ventricular systolic}} dysfunction -Increased {{c1::left ventricular cavity diameter}} -Absence of {{c1::hypertension}}, {{c1::valve}} disease, or significant {{c1::coronary artery}} disease. Also called {{c1::congestive}} cardiomyopathy. Can result from a number of myocardial insults.
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Text Restrictive CardiomyopathyPrimary decrease in ventricular {{c1::compliance}} resulting in impaired {{c1::ventricular filling}} during diastole {{c1::Decreased}} ventricular compliance secondary to {{c1::infiltration}} of the myocardium. May mimic {{c1::constrictive pericarditis}}Caused by:-{{c1::Amyloidosis}} -{{c1::Sarcoidosis}} -{{c1::Storage}} diseases, including {{c1::hemochromatosis}} -Post-radiation {{c1::fibrosis}} -{{c1::Endomyocardial}} disease -{{c1::Metastatic tumors}}
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Text Dilated Cardiomyopathy--Etiology (Genetics)­Genetic factors : familiar in {{c1::30}} to {{c1::50}}% of cases, disease of {{c1::AD}} inheritance (X-linked, AR, and mitochondrial inheritance are less common) ­Genetic abnormalities caused by mutation in over 20 genes encoding genes that encode {{c1::cytoskeletal}} myocyte proteins of the {{c1::sarcolemma}} and {{c1::nuclear membrane}}. ­ Mutations in {{c1::TTN (titin)}}, the largest protein expressed in humans, accounts for up to 20% of all cases of dilated cardiomyopathy. ­ X-linked dilated cardiomyopathy (not a common form) is linked the gene for {{c1::dystrophin}}, the same gene mutated in skeletal myopathies (Duchenne and Becker muscular dystrophies)
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