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33 Cards in this Set
- Front
- Back
- 3rd side (hint)
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define cardiomyopathy
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hrt dz of non-inflamatory nature that results in dysfunction dz of exclusion - RO HTN, congenital malfrm, vlv dyfx, atherosclerosis, pericardial dysfnx
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Name 4 categories of cardiomyopathy
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primary dialated CM, secondary dialated CM, restrictive CM, hypertrophic CM
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secondary CM is secondary to what major classes of dzs
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Metabolic, Neuromuscular, Storage disorders, Infiltrative processes, Immunologic processes
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Kinds of metabolic dzs that lead to secondary CM
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hypo hyperthyroid hypo hyperkalemia nutritional deficit hemochromotosis - later
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Kinds of Neuromuscular dzs that lead to secondary CM
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Friedrich's ataxia MD congenital atrophies
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Storage disorders that lead to secondary CM
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Hunter's-Hurler's synd Glycogen (VPCM) Fabry's (X-linked)
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Infiltrative process that lead to secondary CM
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Leukemia, Carcinomatosis, Sarcoid, Amyloid
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Immunologic process that can lead to secondary DCM
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transplant
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None
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Catagorical causes of primary dialated cardiomyopathy
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Viral, Genetic, Alcohol/toxins
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viruses that cause primary DCM
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cocksackie B, enterovirus
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Of the 4 classification of cardiomyopathy, which one is the most common
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DCM (90%) dialated cardiomyopathy
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is DCM a systolic or diastolic dysfnx
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systolic
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What kind of dysfnx (diastolic/systolic) is HCM
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diastolic
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What class of drugs can cause DCM? give an example
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anthracyclines, doxorubicin
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20 - 30% of DCm are caused by mutations in genes coding for what class of proteins?
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cytoskeletal (No skeleton, so heart dilates, can't stay together)
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Mutations in what different groups of proteins can cause DCM? (4)
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cytoskeletal, dystrophin, mitochondrial, sarcomeric
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What can you say about idiopathic cases of DCM?
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usually fatal
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What is the major problem in HCM?
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abnormal diastolic filling due to thickened vent
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What is the difference in contraction b/w DCM and HCM?
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HCM - powerful, hyperkinetic
DCM - ineffective EF ~25% |
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How is HCM inherited and how many cases are genetic
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AD with variable penetrance, 50% of cases are genetic.
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What kinds of genetic defects can lead to HCM?
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myosin heavy beta chains (1/3), alpha tropomyosin, troponin I and T, myosin light chains
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Is genetic testing valuable in prognosis for HCM? Why or why not
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yes, prognosis varies with defect so pt outcome can be stratified.
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How does HCM present
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exertional dyspnea, harsh systolic ejection murmur
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HCM is a cause of sudden death in young athletes
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Ohhhh reallyyy
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What is Madonna's real name?
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Madonna Louise Veronica Ciccone
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Beside diastolic filling dysfnx, what is another common feature of HCM?
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Ventricular outflow restriction.
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What is the problem in RCM (restrictive cardiomyopathy)
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decrease in ventricular compliance -> decreased vent filling
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HCM and RCM both have problems filling the vent during dia, what is the diff b/w the 2
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HCM forceful systole, due to vent outflow restriction
RCM normal systolic velocity |
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Known causes of RCM (6)?
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endomyocardial fibrosis (most common cause worldwide), Eosinophilic endomyocardial fibrosis (Loffler synd), cardiac amyloidosis, endocardial fibroelastosis, hemochromatyosis, radiation inj.
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The endocardial fibroelastosis cause of RCM preferentially affects a certain age group. What is it?
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younger than 2.
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How does RCM present?
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fatigue, exertional dyspnea, chest pn.
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How does RCM end
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congestive hrt failure
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What similar dz should RCM not be confused with and why?
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constrictive pericarditis - CP is operative.
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