Cardiomyopathy Pathology DS Flashcards
Cardiomyopathy?
describe heart disease resulting from an abnormality in the myocardium
Primary cardiomyopathies?
disease primarily confined to the heart muscle
Secondary cardiomyopathies?
have myocardial invlolvement as a component of a systemic or multiorgan disorder
Categories of Cardiomyopathies?
Dilated cardiomyopathy, Hypertrophic cardiomyopathy, restrictive cardiomyopathy, other causes, left ventricular noncompaction
Common cause of Dilated Cardiomyopathy (DCM)?
Genetic autosomal dominance, gene that encodes dystrophin
Dystrophin?
cell membrane associated cytoskeletal protein that plays a critical role in couping the internal cytoskeleton to the extracellular matrix
mutated in most skeletal myopathies
Other causes DCM?
myocardities, alcohol (beriberi), childbirth
Gross morphology DCM?
enlarged heart, heavy
mural thrombi
mitral reguritation (left or right ventricle dilation)
Mitral reguritation?
when the Left ventricle contracts blood flows back into the left atrium because the mitral valve did not close properly
Micro morphology DCM?
nonspecific and usually do not point to any particular etiologic agent
can be hypertrophied, irregular cells, variable fibrosis present
Clinopathologic pattern of DCM?
Same no matter the cause
end stage- ejection fraction less than 25%, 50% die within 2 years
secondary mitral regurgitation and abnormal cardiac and abnormal cardiac rhythms are common
death from progressive cardiac failure or arrthymia, embolism from thrombus can occur
DCM is primarily?
impairment of contractility
treat DCM?
cardiac transplant, long term ventricular assist
Arrhythmogenic right ventricular cardiomypathy (ARVC)?
under the category of DCM
inherited disease of cardiac muscle that causes right ventricular failure and various rhythm disturbances (ventricular tachycardia or fibrilation)
– sudden death in young people
ARVC cause?
defective cell adhesion protein in desmosome that link adjacent cardiac myocytes
Naxos syndrome?
disorder characterized by ARVC and hyperkeratosis of plantar palmar skin surfaces that is associated with mutation in gene encoding plakoglobin
Hypertrophic cardiomyopathy? (HCM)
myocardial hypertrophy, poorly compliant left ventricle mycardium leading to abnomal diastolic filling, and sometimes intermittent ventricular outflow obstruction
HCM summary?
impairment of compliance (diastolic dysfuntion)
thick walled, heavy, hypercontracting
Common cause HCM?
single gene mutation
most common cardiovascular problem caused by single gene mutation
genes encoding proteins on the sarcomere
Most common gene/protein in HCM?
gene encoding Beta myosin heavy chain (most)
cardiac TnT, alpha tropomyosin, myosin binding protein C
Common btw DCM and HCM?
hypertrophy, dilation, fibrosis, intersitial
lead to heart failure, sudden death, atrial fibrilation, stroke
DIfference DCM and HCM?
HCM has a thick walled, heavy, hypercontracting heart
HCM is caused by mutations in the sarcomere while DCM is a disease caused my abnormalities of cytoskeletal proteins
DCM is flabby, hypercontracting
Gross morph of HCM?
massive myocardial hypertrophy, asymmetrical is more common
thickened ventricular septum
endocardial thickening, mural plaque formation in left ventricular outflow tract and thickening of anterior mitral leaflet– echo functional left ventricular outflow tract obstruction during midsystole
histo findings HCM?
extensive myocyte hypertrophy
haphazard disarray of bundles of myocytes, individual myocytes, and contractile elements in sarcomeres within cells
intersitial and replacement fibrosis
