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33 Cards in this Set

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