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RC Test 4 > B4.069 Cardiomyopathies > Flashcards

B4.069 Cardiomyopathies Flashcards

1
Q

definition of cardiomyopathy

A

heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that are frequently genetic
can be confined to heart or part of a generalized systemic disorder
often lead to cardiovascular death or progressive heart failure related disability

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2
Q

normal heart weight

A

female: 250-300 g
male: 300-350 g

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3
Q

RV wall thickness

A

0.3 to 0.5 cm

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4
Q

LV wall thickness

A

1.3 to 1.5 cm

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5
Q

hypertrophy

A

grater heart weight or ventricular thickness

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6
Q

dilation

A

enlarged chamber size

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7
Q

cardiomegaly

A

increase in cardiac weight or size

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8
Q

5 classes of cardiomyopathy

A
  1. dilated (congestive)
  2. hypertrophic
  3. restrictive (infiltrative)
  4. arrhythmogenic
  5. unclassified
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9
Q

most common cardiomyopathy

A

dilated

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10
Q

definition of dilated cardiomyopathy

A

LV or biventricular dilation, impaired contractility, eventual congestive heart failure

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11
Q

pathogenesis of dilated cardiomyopathy

A 
genetic factors most common
viral myocarditis
alcohol, chemo, cobalt, cocaine, vasopressors
iron overload
immunologic abnormailities
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12
Q

how do genetic factors cause dilated cardiomyopathy?

A

defects in force transmission lead to dilatation, poor contractility

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13
Q

gross pathology of dilated cardiomyopathies

A 
cardiomegaly (>500-900 g)
globoid appearance
hypertrophy and dilatation
flabby myocardium, wall collapses
subendocardial scars
mural thrombi, apex of LV, emboli
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14
Q

non specific gross pathology of cardiomyopathies

A

atrophy and hypertrophy of myocardial fibers

interstitial and perivascular fibrosis, subendocardial fibrosis

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15
Q

electron microscopy findings in cardiomyopathy

A

increased mitochondria and loss of sarcomeres

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16
Q

what are some causes of secondary dilated cardiomyopathies

A

toxic (ethanol, cobalt, catecholamines, etc)
pregnancy (last trimester, 1st 6 mo after delivery)
viral cardiomyopathy

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17
Q

clinical features of dilated cardiomyopathies

A 
asymptomatic LV dilatation
progressive exercise intolerance
CHF
75% die within 5 yrs
transplantation
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18
Q

definition of hypertrophic cardiomyopathies

A 
L or L/RV hypertrophy and structural derangement
asymmetric hypertrophy
hypertrophic subaortic stenosis
obstructive cardiomyopathy
1:500, genetic factors
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19
Q

genetic factors which can contribute to hypertrophic cardiomyopathies

A

100 mutations in at least 9 genes encoding proteins of the sarcomere
defects in force generation lead to HCM

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20
Q

most common mutations in hypertrophic cardiomyopathies

A
  1. beta-myosin heavy chain 25%
  2. myosin binding protein C 25%
  3. cardiac troponin I 3-5%
  4. cardiac troponin T 3-5%
  5. alpha-tropomyosin 1%
  6. myozenin 2 0.04%
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21
Q

gross pathology of hypertrophic cardiomyopathies

A 
cardiomegaly
asymmetric myocardial hypertrophy
septum/free wall > 1.5 cm
obstructive hemodynamic pattern
LV thick wall, small cavity, myocardial fibrosis
mitral valve floppy and dysplastic
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22
Q

genetic disorders associated with hypertrophic cardiomyopathy

A

Noonan syndrome
Lentiginosis
Costello syndrome
Mitochondrial hypertrophies

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23
Q

exaggerated hypertrophies (not cardiomyopathy but on differential)

A

hypertensive LVH
athletes heart
neonatal cardiomegaly

24
Q

clinical features of hypertrophic cardiomyopathies

A 
asymptomatic 
screening of family
sudden death
 dyspnea, angina, syncope
unsuspected HCM at autopsy of sudden death in a young competitive athlete
25
Q

treatment of hypertrophic cardiomyopathy

A

B blockers
Ca channel blockers
surgery

26
Q

definition of restrictive cardiomyopathies

A

a group of diseases in which myocardial or endocardial abnormalities limit diastolic filling, while allowing contractile function to remain relatively normal
least common form

27
Q

2 groups of restrictive cardiomyopathies

A

endocardial

myocardial

28
Q

3 conditions with endomyocardial involvement

A

endomyocardial fibrosis
loeffler endocarditis
endocardial fibroelastosis

29
Q

endomyocardial fibrosis

A

equatorial Africa
CHF
death
children and young adults

30
Q

loeffler endocarditis

A 
temperate zones
CHF
death
men in 5th decade
hypereosinophelia, myeloproliferative disorder
31
Q

endocardial fibroelastosis

A

focal or diffuse thickening, LV

first 2 years of life

32
Q

pathology of endomyocardial diseases

A

grayish white thickened endocardium
mural thrombi
fibrotic endocardium

33
Q

conditions with predominant myocardial involvement

A

amyloidosis
sarcoidosis
storage disease
idiopathic

34
Q

amyloidosis pathology

A

cardiomegaly

amyloid infiltrate

35
Q

clinical presentation of amyloidosis

A

common cause of death in patients with MM
R sided heart failure
arrhythmias

36
Q

senile cardiac amyloidosis

A

elderly patients

transthyretin deposition-better prognosis

37
Q

staining for cardiac amyloidosis

A

congo red

apple green birefringences

38
Q

sarcoidosis

A

generalized granulomatous disease

non-caseating granulomas, fibrosis

39
Q

clinical presentation of sarcoidosis

A

mixed pattern of dilated or restrictive cardiomegaly
CHF
arrhythmias
sudden death

40
Q

examples of storage diseases

A 
glycogen storage diseases (pompe, cori, Anderson)
mucopolysaccharidoses
sphingolipidoses (fabry, gaucher)
iron storage (hemochromatosis)
41
Q

what happens in storage diseases

A

protein cannot be degraded and gets stored in muscle

42
Q

results of hemochromatosis

A

Fe deposition
primary or secondary
CHF
dilated heart, brown color, LVH, fibrosis

43
Q

imaging of hemochromatosis

A

dilated brown heart

blue dots on Perl’s iron stain

44
Q

definition of arrhythmogenic cardiomyopathy

A

primary myocardial disease characterizes by progressive adipose of fibroadipose replacement of ventricular myocardium
“RV arrhythmogenic dysplasia”

45
Q

gross pathology of arrhythmogenic cardiomyopathy

A 
cardiomegaly
fatty infiltration of RV myocardium
partial or transmural
occasionally biventricular involvement
mural thrombi
46
Q

histopath findings of arrhythmogenic cardiomyopathy

A

fatty or fibrofatty infiltration of myocardium

47
Q

pathogenesis of arrhythmogenic cardiomyopathy

A 
congenital defect (aplasia of myocardium)
genetic factors (20-30% family inheritance, mitochondrial DNA mutations)
acquired factors (chronic myocarditis, immune, viral)
48
Q

clinical features of arrhythmogenic cardiomyopathy

A

concealed form
overt electrical heart disorder, typical presentation
RV failure
biventricular failure, late

49
Q

myocarditis

A

diverse group of pathologic entities
infections
immune related (postviral, poststreptococcal, SLE)
unknown (sarcoid, giant cell myocarditis)

50
Q

viral myocarditis

A

lymph myocarditis + myocyte injury

51
Q

Chagas disease

A

trypanosoma cruzi

52
Q

hypersensitivity myocarditis

A

inflame infiltrate + eosinophilia

53
Q

giant cell myocarditis

A

inflame infiltrate + giant cells + myocyte injury

54
Q

clinical features of myocarditis

A 
heart failure
arrhythmias, dyspnea, palpitations
dilated cardiomyopathy
sudden death
symptoms related to primary disease
55
Q

specific caridomyopathies

A

muscular dystrophies can have cardiac manifestations
neuromuscular disorders
histiocytoid cardiomyopathy
ischemic cardiomyopathy (common)

RC Test 4 (22 decks)

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