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Classification done by Echo. 3 patterns: 1. dilated cardiomyopathy (dilation of L ventricle w ejection fraction of < 40% so it's systolic dysfunction). 2. hypertrophic cardiomyopathy (Left Ventricular hypertrophy w preserve EF - diastolic disfunction with filling. 3. restrictive cardiomyopathy (normal left ventricle associated with reduced filling - diastolic dysfunction)
Dilated cardiomyopathy: Hereditary - defects in cytoskeleton protein. 1. Titin 2. dystrophin. Present with CHF in middle age. SOB, edema. Diagnosis of exclusion. Positive family history. Infectious myocarditis by viruses is most common here. aggressive viruses: coxsachie B, adenovirus, parvoB19 = all involve multiple systems. RASH. Pathophys: direct injury vs immune. adaptive immunity = chronic inflammatory cells. Natural Hx is benign self limited. Dx: evidence of systemic virus, evidence of myocete cell death (CKMB or troponin), gold standard is heart biopsy.
Hypertrophic cardiomyopathy: genetic abnormality in 2/3rds. Clinical presentation is asymptomatic: find stuff by accident. Symptomatic: CHF, chest pain, arrhythmia. Pathophys: myocyte hypertrophy, LVH -focal and upper septum (near bundle of HIS), Left ventricular outlet tract abnormalities (decreased filling, abnormal arrhythmias, abnormal myocyte function. Physical exam: systolic murmur. normal murmur increase with increase flow, in HCM the murmur increases with DECREASED flow! Rx - beta blocker, implantable defibrillator
Restrictive cardiomyopathy: normal LV w less filling (normal ejection fraction), bilateral atrial enlargement. Clinical presentation: SOB, R sided edema. Pathophysiology: infiltration - fibrosis (Scleroderma, RA) - hemochromatosis (iron) - amyloidosis familial conditions; fibrosis - less ventricular filling. Dx: diagnosis of exclusion - or cardiac biopsy
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