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RC Test 4 > B4.069 - Cardiomyopathies > Flashcards

B4.069 - Cardiomyopathies Flashcards

1
Q

what immunologic abnormalitie can cause dilated cardiomyopathy

A

mitochondrial and myocardial auto Abs

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2
Q

gross pathology of hypertrophic cardiomyopathy

A

asymmetric myocardial hypertrophy (septum)

septum/free wall >1.5

obstructive hemodynamic pattern

LV thick wall, small cavity, myocardial fibrosis

mitral valve (floppy, dysplastic)

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3
Q

what is a dilated cardiomyopathy

A

left ventricular or diventricular dilation, impaired contractility and eventually congestive heart failure

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4
Q

pathogenesis of arrhythmogenic cardiomyopathy

A

congenital defect

genetic factors - 20-30% familial inheritance, AD

mitochondrial DNA mutatiosn, etc.

acquired factors - chronic myocarditis

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5
Q

what is loeffler endocarditis associated with

A

temperate zones, CHF, death, men 5th decade

hypereosinophilia, myeloproliferative disorder

Chr rearrang PDGFR alpha and beta genes, TK inhibitors

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6
Q

describe cardiomyopathy of pregnancy

A

last trimester

more common in multiparous women, over 30, african american

>50% spontaneous recovery

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7
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

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8
Q

things associated with endocardial fibroelastosis

A

focal or diffuse thickening, LV

first 2 years of life

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9
Q
A

endomyocardial disease in restrictive cardiomyopathy

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10
Q

what is myocarditis

A

a diverse group of pathogenic entities

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11
Q

pathologic findings of viral myocarditis, chagas, HS, giant cell

A
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12
Q

definition of cardiomyopathy

A

heart muscle disease of unknown cause

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13
Q

most common mutations leading to hypertrophic cardiomyopathy

A

beta myosin heavy chain - 25%

myosin binding protein C - 25%

cardiac troponin I - 3-5%

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14
Q

stages of endomyocardial diseases

A

necrotic

thrombotic

fibrotic

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15
Q

conditions with predominant myocardial involvement

A

amyloidosis

sarcoidosis

storage diseases

idiopathic

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16
Q
A

sarcoidosis

non caseating granuloma

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17
Q
A

fatty infiltration of myocardium

arrhythmogenic cardiomyopathy

restrictive

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18
Q

hypertrophic cardiomyopathy pathogenesis

A

genetic factors, mutations in at least 9 genes encoding proteins of the sarcomere

defects in force generation lead to HCM

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19
Q

definition of hypertrophic cardiomyopathy

A

L or L/RV hypertrophy and structural derangement

asymmetric septal hypertrophy

hypertrophic muscular subaortic stenosis

obstructive cardiomyopathy

1:500 genetic factors

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20
Q
A

dilated cardiomyopathy

globoid appearance

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21
Q

Rx for hypertrophic cardiomyopathy

A

beta blockers

Ca channel blockers

surgery

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22
Q
A

left normal

right atrophy and fibrosis

dilated cardiomyopathy

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23
Q

what are the classifications of cardiomyopathy according to the Am heart association

A

genetic - hypertrophic, arrythmogenic, glycogen storage, mitochondrial defects

mixed - dilated, restrictive

acquired - inflammatory, peripartum, stress related

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24
Q

cardiomegaly

A

increased cardiac weight or size

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25
histo of arrhythmogenic cardiomyopathy
restricrive fatty or fibrofatty infiltration fo myocardium
26
2 groups of restrictive cardiomyopathy
endocardial, myocardial
27
myofiber disarray hypertrophic cardiomyopathy
28
electron microscopy of dilated cardiomyopathy
increased mitochondria and loss of sarcomeres
29
dilated cardiomyopathy 2 D's
30
differential Dx for hypertrophic cardiomyopathy
genetic disorders - noonan syndrome, lentiginosis, costello syndrome, mitochondrial exaggerated hypertrophies - hpyertensive LVH, athletes heart, neonatal cardiomegaly
31
right ventricle thickness and left ventricle thickness
right - .3-.5 left - 1.3 -1.5
32
how does dilation occur
defect in force transmission lead to dilation, poor contractility
33
what viruses can cause dilated cardiomyopathy
coxsakie B
34
cardiac sarcoidosis non caseating granuloma
35
normal heart weigth
females - 250 - 300 males - 300 - 350
36
hemochromatosis (iron storage diesease) left - dilated brown heart right - perls iron stain restrictive cardiomyopathy
37
mural thrombi in endomyocardial diseases in restrictive cardiomyopathy
38
arrhythmogenic cardiomyopathy gross pathology
cardiomegaly fatty infiltration of RV myocardium partial or transmural occasionally biventricular mural thrombi
39
what exposures can lead to dilated cardiomyopathy
alcohol, chemo, cobalt, cocaine, vasopressors, iron overload
40
conditios with endomyocardial involvement in restrictive cardiomyopathy
endomyocardial fibrosis loeffler endocarditis endocardial fibroelastosis
41
pathology of endomyocardial diseases in restrictive cardiomyopathy
grayish, white thickened endocardium mural thrombi fibrotic endocardium
42
cardiac amyloidosis congo red - left apple green birefringence - right
43
what is sarcoidosis and its pathology/clinically
generalized granulomatous disease non caseating granulomas, fibrosis clinically - mixed pattern of dilated or restricteve cardiomegaly, CHF, arrhythmias, sudden death
44
pompe disease glycogen storage disease restrictive cardiomyopathy
45
myofiber disarray hypertrophic cardiomyopathy
46
amyloidosis pathology
cardiomegaly, amyloid infiltrate common cause of death in paitients with MM clinically R-sided failure, arrhythmias senile cardiac amyloidosis - elderly patients transthyretin deposition better prognosis
47
what is endomyocardial fibrosis associated with
equatorial africa, CHF, death, children and young adults
48
causes of toxic dilated cardiomyopathy
ethanol, cobalt, catecholamines, anthracyclines, cyclophosphamide, cocaine
49
restrictive cardiomyopathy
50
clinical features of arrrhythmogenic cardiomyopathy
concealed form overt electrical heart disorder, typical presentation RV failure Biventricular failure, late
51
IVS asymmetric hypertrophy Hypertrophic cardiomyopathy
52
iron storage disease features
restrictive cardiomyopathy Fe deposition primary or secondary CHF Dilated heart, brown color, LVH, fibrosis
53
what are types of storage diseases causing restrictive cardiomyopathy
glycogen storage diseases - pompe, cori, anderson mucoplysaccharidoses sphingolipidoses - fabry disease, faucher disease
54
etiology and pathogenesis of myocarditis
infections - viruses, chlamydiae, bacteria. protozoa, helminths immune related - post viral, poststrep, SLE, drug HS, transplant rej. unkonw - sarcoidosis. giant cell myocarditis
55
fabry disease glycosphingolipid storage disease restrictive cardiomyopathy
56
what is arrhythmogenic cardiomyopathy
primary mitochondrial disease characterized by progressive adipose or fibroadipose replacement of ventricular myocardium RV arrhythmogenic dysplasia
57
cardiac sarcoidosis
58
arrhythmogenic cardiomyopathy
59
fibrosis seen in endomyocardial diseases in restrictive cardiomyopathy
60
most common type of cardiomyopathy
dilated
61
specific cardiomyopathies (restrictive)
muscular dystrophy - duchene, becker, mytonic neuromuscular disorders and mypathies - mitochondrial, friedreich ataxia histiocytoid cardiomyopathy ischemic cardiomyopathy
62
clinical features of hypertrophic cardiomyopathy
asymptomatic screening of family of HCM patient sudden death dyspnea, angina, syncope unsuspected HCM at autopsy of sudden death in young competitive athlete
63
gross pathology of dilated cardiomyopathy
atrophy and hypertrophy of myocardial fibers interstitial and perivascular fibrosis, subendocardial fibrosis
64
clinical features of myocarditis (restrictive)
HF arrhythmias, dyspnea, palpitation dilated cardiomyopathy sudden death symptoms related to primary disease
65
clinical features of dilated cardiomyopathy
asymptomatic LV dilation progressive exercise intolerance CHF 76% die within 5 years transplantation
66
pathology of dilated cardiomyopathy
cardiomegaly globoid appearance, 4 chamber dilation hypertrophy and dilation flabby myocardium, wall collapses subendocardial scars mural thrombi, apex of LV, emboli

RC Test 4 (10 decks)

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