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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
Q

histo of arrhythmogenic cardiomyopathy

A

restricrive

fatty or fibrofatty infiltration fo myocardium

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26
Q

2 groups of restrictive cardiomyopathy

A

endocardial, myocardial

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27
Q
A

myofiber disarray

hypertrophic cardiomyopathy

28
Q

electron microscopy of dilated cardiomyopathy

A

increased mitochondria and loss of sarcomeres

29
Q
A

dilated cardiomyopathy

2 D’s

30
Q

differential Dx for hypertrophic cardiomyopathy

A

genetic disorders - noonan syndrome, lentiginosis, costello syndrome, mitochondrial

exaggerated hypertrophies - hpyertensive LVH, athletes heart, neonatal cardiomegaly

31
Q

right ventricle thickness and left ventricle thickness

A

right - .3-.5

left - 1.3 -1.5

32
Q

how does dilation occur

A

defect in force transmission lead to dilation, poor contractility

33
Q

what viruses can cause dilated cardiomyopathy

A

coxsakie B

34
Q
A

cardiac sarcoidosis non caseating granuloma

35
Q

normal heart weigth

A

females - 250 - 300

males - 300 - 350

36
Q
A

hemochromatosis (iron storage diesease)

left - dilated brown heart

right - perls iron stain

restrictive cardiomyopathy

37
Q
A

mural thrombi in endomyocardial diseases in restrictive cardiomyopathy

38
Q

arrhythmogenic cardiomyopathy gross pathology

A

cardiomegaly

fatty infiltration of RV myocardium

partial or transmural

occasionally biventricular

mural thrombi

39
Q

what exposures can lead to dilated cardiomyopathy

A

alcohol, chemo, cobalt, cocaine, vasopressors, iron overload

40
Q

conditios with endomyocardial involvement in restrictive cardiomyopathy

A

endomyocardial fibrosis

loeffler endocarditis

endocardial fibroelastosis

41
Q

pathology of endomyocardial diseases in restrictive cardiomyopathy

A

grayish, white thickened endocardium

mural thrombi

fibrotic endocardium

42
Q
A

cardiac amyloidosis

congo red - left

apple green birefringence - right

43
Q

what is sarcoidosis and its pathology/clinically

A

generalized granulomatous disease

non caseating granulomas, fibrosis

clinically - mixed pattern of dilated or restricteve cardiomegaly, CHF, arrhythmias, sudden death

44
Q
A

pompe disease

glycogen storage disease

restrictive cardiomyopathy

45
Q
A

myofiber disarray

hypertrophic cardiomyopathy

46
Q

amyloidosis pathology

A

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
Q

what is endomyocardial fibrosis associated with

A

equatorial africa, CHF, death, children and young adults

48
Q

causes of toxic dilated cardiomyopathy

A

ethanol, cobalt, catecholamines, anthracyclines, cyclophosphamide, cocaine

49
Q
A

restrictive cardiomyopathy

50
Q

clinical features of arrrhythmogenic cardiomyopathy

A

concealed form

overt electrical heart disorder, typical presentation

RV failure

Biventricular failure, late

51
Q
A

IVS asymmetric hypertrophy

Hypertrophic cardiomyopathy

52
Q

iron storage disease features

A

restrictive cardiomyopathy

Fe deposition

primary or secondary

CHF

Dilated heart, brown color, LVH, fibrosis

53
Q

what are types of storage diseases causing restrictive cardiomyopathy

A

glycogen storage diseases - pompe, cori, anderson

mucoplysaccharidoses

sphingolipidoses - fabry disease, faucher disease

54
Q

etiology and pathogenesis of myocarditis

A

infections - viruses, chlamydiae, bacteria. protozoa, helminths

immune related - post viral, poststrep, SLE, drug HS, transplant rej.

unkonw - sarcoidosis. giant cell myocarditis

55
Q
A

fabry disease

glycosphingolipid storage disease

restrictive cardiomyopathy

56
Q

what is arrhythmogenic cardiomyopathy

A

primary mitochondrial disease characterized by progressive adipose or fibroadipose replacement of ventricular myocardium

RV arrhythmogenic dysplasia

57
Q
A

cardiac sarcoidosis

58
Q
A

arrhythmogenic cardiomyopathy

59
Q
A

fibrosis seen in endomyocardial diseases in restrictive cardiomyopathy

60
Q

most common type of cardiomyopathy

A

dilated

61
Q

specific cardiomyopathies (restrictive)

A

muscular dystrophy - duchene, becker, mytonic

neuromuscular disorders and mypathies - mitochondrial, friedreich ataxia

histiocytoid cardiomyopathy

ischemic cardiomyopathy

62
Q

clinical features of hypertrophic cardiomyopathy

A

asymptomatic

screening of family of HCM patient

sudden death

dyspnea, angina, syncope

unsuspected HCM at autopsy of sudden death in young competitive athlete

63
Q

gross pathology of dilated cardiomyopathy

A

atrophy and hypertrophy of myocardial fibers

interstitial and perivascular fibrosis, subendocardial fibrosis

64
Q

clinical features of myocarditis (restrictive)

A

HF

arrhythmias, dyspnea, palpitation

dilated cardiomyopathy

sudden death

symptoms related to primary disease

65
Q

clinical features of dilated cardiomyopathy

A

asymptomatic LV dilation

progressive exercise intolerance

CHF

76% die within 5 years

transplantation

66
Q

pathology of dilated cardiomyopathy

A

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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