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CARDIOMYOPATHY Flashcards

1
Q

The term cardiomyopathy (literally, _______) by convention is used to describe _____ resulting from a __________

A

heart muscle disease

heart disease

primary abnormality in the myocardium.

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2
Q

In many cases, cardiomyopathies are idiopathic (i.e., of unknown cause)

T/F

A

T

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3
Q

well-defined myocardial diseases may, in the end, resemble those without known causes, both functionally and structurally.

T/F

A

T

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4
Q

Major advance in our understanding has given _____ of many myocardial diseases, previously considered idiopathic.

A

genetic basis

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5
Q

etiologic distinctions of cardiomyopathies have become somewhat blurred

T/F

A

T

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6
Q

Three clinical, functional, and pathologic patterns :

______ cardiomyopathy (______)

_______ cardiomyopathy (____)

________ cardiomyopathy
.

A

Dilated ; DCM

Hypertrophic; HCM

Restrictive

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7
Q

commonest (___ %) cardiomyopathy is ?????

________ cardiomyopathy- least prevalent.

A

90

Dilated cardiomyopathy (DCM)

Restrictive

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8
Q

_________ are used widely in the diagnosis of cardiomyopathies

A

Endomyocardial biopsies

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9
Q

Dilated cardiomyopathy (DCM):

Left ventricular ejection fraction: ___

Mechanisms of heart failure:____

A

Less than 40%

Impairment of contraction( systolic dysfunction)

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10
Q

Hypertrophic cardiomyopathy (HCM):

Left ventricular ejection fraction: ___

Mechanisms of heart failure:____

A

50-80%

Impairment of compliance ( diastolic dysfunction)

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11
Q

Restrictive cardiomyopathy:

Left ventricular ejection fraction: ___

Mechanisms of heart failure:____

A

45-90%

Impairment of compliance ( diastolic dysfunction)

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12
Q

DILATED CARDIOMYOPATHY

DCM is applied to a form of cardiomyopathy characterized by progressive cardiac ______,______, and _______ (______) dysfunction.

A

hypertrophy, dilation, and contractile

systolic

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13
Q

DILATED CARDIOMYOPATHY

It is sometimes called ______ cardiomyopathy.

A

congestive

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14
Q

DILATED CARDIOMYOPATHY

Myocarditis: Viral nucleic acids from ________ and other ____viruses have been detected in the myocardium of some patients, and sequential _____ biopsies have demonstrated progression from ——— to _______

A

coxsackievirus B

entero; endomyocardial

myocarditis to DCM

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15
Q

DILATED CARDIOMYOPATHY

Alcohol or other toxicity: Alcohol abuse is also strongly associated with the development of DCM, raising the possibility that __________ or ________ may be the cause of the myocardial injury.

A

ethanol toxicity or a secondary nutritional disturbance

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16
Q

DILATED CARDIOMYOPATHY

Pregnancy-associated: A special form of DCM, termed ______ cardiomyopathy, occurs (early or late?) in pregnancy or ____ to _____ ————.

A

peripartum

Late

several weeks to months

postpartum

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17
Q

The cause of peripartum cardiomyopathy is (well or poorly?) understood

A

F

The cause of peripartum cardiomyopathy is poorly understood but is probably multifactorial

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18
Q

DILATED CARDIOMYOPATHY

peripartum cardiomyopathy

Pregnancy-associated _______, ______ overload, ______ deficiency, other metabolic derangement, or as yet poorly characterized _______ reaction may be involved.

A

hypertension

volume

nutritional

immunologic

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19
Q

DILATED CARDIOMYOPATHY

Genetic : Genetic influences have been documented in some cases, particularly when _____________________

A

multiple members of a family are affected.

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20
Q

DILATED CARDIOMYOPATHY

DCM has a familial occurrence in ___ to ____ % of cases.

A

20 to 30

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21
Q

DILATED CARDIOMYOPATHY
MORPHOLOGY.

Nevertheless, because of the wall ______ that accompanies _____, the ventricular wall thickness may be ____________________________ than normal.

A

thinning

dilation

less than, equal to, or more

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22
Q

In DCM, the heart is usually not heavy

T/F

A

F

It’s heavy

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23
Q

DILATED CARDIOMYOPATHY
MORPHOLOGY.

In DCM, the heart is usually heavy, weighing _______ times normal, and large and ______, with ____ of ____ chambers.

A

two to three

flabby

dilation

all

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24
Q

DILATED CARDIOMYOPATHY

______ thrombi are common and may be a source of _______

Primary valvular alterations are (present or absent?)

mitral or tricuspid regurgitation when present are a result of ___________ (______ regurgitation).

A

Mural; thrombo-emboli.

Absent

left ventricular chamber dilation

functional

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25
Q

DILATED CARDIOMYOPATHY

The coronary arteries are usually ________________________, but any coronary arterial obstructions that do exist are (sufficient or insufficient?) to explain the degree of cardiac dysfunction.

A

free of significant narrowing

Insufficient

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26
Q

In DILATED CARDIOMYOPATHY

The coronary arteries are usually free of significant narrowing

T/F

A

T

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27
Q

DILATED CARDIOMYOPATHY: The histologic abnormalities in idiopathic DCM are :

-nonspecific

  • usually reflect a specific causative agent.

T/F

A

T

F(usually do not reflect a specific causative agent.)

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28
Q

DILATED CARDIOMYOPATHY: The histologic abnormalities in idiopathic DCM

•Their severity reflects the degree of dysfunction

•the severity indicates the patient’s prognosis.

T/F

A

F

F

Their severity does not necessarily reflect the degree of dysfunction or the patient’s prognosis.

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29
Q

DILATED CARDIOMYOPATHY: The histologic abnormalities in idiopathic DCM

Most muscle cells are ______ with ______ nuclei, but many are _______ or _______

A

hypertrophied

enlarged

attenuated or stretched.

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30
Q

In DILATED CARDIOMYOPATHY

Interstitial and endocardial fibrosis of variable degree is present

Large subendocardial scars replace individual cells or groups of cells

T/F

A

T

F(small)

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31
Q

In DILATED CARDIOMYOPATHY

small subendocardial scars replace individual cells or groups of cells, probably reflecting ______________________ caused by ______- induced imbalance between ______ and ———

A

healing of previous secondary myocyte ischemic necrosis

hypertrophy

perfusion supply and demand.

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32
Q

DILATED CARDIOMYOPATHY
Clinical Features.

DCM may occur at any age

DCM Does not occur in childhood

T/F

A

T

F( it does)

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33
Q

DILATED CARDIOMYOPATHY
Clinical Features.

It presents with (slowly or rapidly?) progressive _________, but patients may slip precipitously from a _______ to _________ state.

A

Slowly

congestive heart failure

compensated to a decompensated functional

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34
Q

DILATED CARDIOMYOPATHY
Clinical Features.

In the end stage, patients often have ejection fractions of (more or less ?) than ___% .

A

Less

25

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35
Q

DCM commonly affects those ___-____ years old.

A

20 to 60

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36
Q

normal left ventricular ejection fraction is approximately ___ to ____%

A

50 to 65

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37
Q

DILATED CARDIOMYOPATHY

Fifty percent of patients die within ________, and only ___% survive longer than ___ years.

Death is usually attributable to progressive _______ or ______

A

2 years

25; 5

cardiac failure or arrhythmia.

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38
Q

Embolism from dislodgment of an intracardiac thrombus may occur in DCM

T/F

A

T

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39
Q

DILATED CARDIOMYOPATHY

___________ is frequently recommended as a fix

A

Cardiac transplantation

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40
Q

A variant of ___________________ is arrhythmogenic right ventricular dysplasia

A

DCM

41
Q

Arrhythmogenic Right Ventricular
Cardiomyopathy

Sometimes a familial disorder that is most commonly associated with (left or right?) -sided and sometimes (left or right?) -sided ______ and various ______ disturbances, particularly ____________

A

right

Left

heart failure; rhythm

ventricular tachycardia.

42
Q

In Arrhythmogenic Right Ventricular
Cardiomyopathy , Sudden death may occur.

T/F

A

T

43
Q

In Arrhythmogenic Right Ventricular
Cardiomyopathy

Morphologically the (left or right?) ventricular wall is severely ______ because of ________, with extensive ____ infiltration and interstitial ______.

A

Right

thinned; loss of myocytes

fatty; fibrosis

44
Q

In Arrhythmogenic Right Ventricular
Cardiomyopathy

Although a gene defect was localized on chromosome _____, the pathogenesis remains _____.

A

14

obscure

45
Q

HCM is also known by such terms as ______________ and ______________ cardiomyopathy.

A

idiopathic hypertrophic subaortic stenosis

hypertrophic obstructive

46
Q

HCM

It is characterized by ________, abnormal __________, and, in about one third of cases, intermittent _____________

A

myocardial hypertrophy

diastolic filling

left ventricular outflow obstruction.

47
Q

HCM

The heart is (light or heavy?) , (muscular or flabby?) , and (hypo or hyper?) - contracting, in striking contrast to the (muscular or flabby?) , and (hypo or hyper?) - contracting heart of DCM.

A

Heavy; Muscular ; hypo

Flabby; hypo

48
Q

HCM is primarily a (systolic or diastolic?) rather than (systolic or diastolic?) disorder.

A

Diastolic

Systolic

49
Q

HCM morphology

The essential feature is massive ______ without __________

A

myocardial hypertrophy

ventricular dilation.

50
Q

HCM MORPHOLOGY

The classic pattern is said to be _______ thickening of the _______ as compared with the free wall of the left ventricle (with a ratio >1.3), frequently termed _________________.

A

disproportionate

ventricular septum

asymmetric septal hypertrophy

51
Q

HCM MORPHOLOGY

In about ____% of cases, however, the hypertrophy is symmetrical.

A

10

52
Q

HCM MORPHOLOGY

On cross-section, the ventricular cavity loses its usual __________ shape and may be compressed into a _________ configuration by bulging of the ______ into the ______.

A

round-to-ovoid

banana-like

ventricular septum

lumen

53
Q

HYPERTROPHIC CARDIOMYOPATHY

Although disproportionate hypertrophy can involve the entire septum, it is usually most prominent in the ______

A

subaortic region.

54
Q

HYPERTROPHIC CARDIOMYOPATHY

Also often present are _____ thickening or _____ formation in the _________ and thickening of the ________ leaflet;

A

endocardial

mural plaque

left ventricular outflow tract

anterior mitral

55
Q

HYPERTROPHIC CARDIOMYOPATHY

endocardial thickening or mural plaque formation in the left ventricular outflow tract and thickening of the anterior mitral leaflet!

Both findings are a result of contact of the _________ with the _____ during _______ correlating with the echocardiographic functional ___________ obstruction during _________.

A

anterior mitral leaflet

septum

ventricular systole

left ventricular outflow tract

midsystole

56
Q

HYPERTROPHIC CARDIOMYOPATHY

____-stage ventricular _____ may occur (early or late?) in a (minority or majority ?) of patients.

A

End; dilation

Late

Minority

57
Q

HYPERTROPHIC CARDIOMYOPATHY

The most important histologic features of the myocardium in HCM are

((1))extensive myocyte _____ to a degree unusual in other conditions, with transverse myocyte diameters frequently more than ____ mum (normal approximately ___ mum)

A

hypertrophy

40; 15

58
Q

HYPERTROPHIC CARDIOMYOPATHY: The most important histologic features of the myocardium in HCM are

(2) haphazard disarray of ____________,__________, and ____________ in sarcomeres within cells ( myofiber disarray)

A

bundles of myocytes, individual myocytes, and contractile elements

59
Q

HYPERTROPHIC CARDIOMYOPATHY: The most important histologic features of the myocardium in HCM are

(3) interstitial and replacement ______.

A

fibrosis

60
Q

The two most common diseases that must be distinguished from HCM are _______ and other infiltrative/deposition disorders and ______ heart disease coupled with age- related __________

Occasionally, ________ or _________ can also mimic HCM.
•

A

amyloidosis

hypertensive

subaortic septal hypertrophy.

valvular or congenital subvalvular aortic stenosis

61
Q

HYPERTROPHIC CARDIOMYOPATHY: PATHOGENESIS.

In approximately half of patients, the disease is ______, and the pattern of transmission is _______ with variable expression.

Remaining cases appear to be ______; it is possible that some represent new mutations or are the result of ___________ transmission with reduced ______.

A

familial; autosomal dominant

sporadic

autosomal recessive

gene penetrance

62
Q

HCM

The genetic defects in HCM comprise mutations in any one of four genes that encode proteins of the cardiac contractile elements, the _________:

_________
_______
_________
__________

A

sarcomeres

beta-myosin heavy chain

cardiac troponin T

alpha-tropomyosin

myosin-binding protein C.

63
Q

The genetic defects in HCM comprise mutations in any one of four genes that encode proteins of the cardiac contractile elements, the sarcomeres, with ___________ being the most frequently affected

A

beta-myosin heavy chain

64
Q

HCM

More than ____ distinct mutations in these proteins have been identified in HCM, and the molecular defect responsible for unrelated cases varies greatly.

A

50

65
Q

HYPERTROPHIC CARDIOMYOPATHY CLINICAL FEATURES.

The basic physiologic abnormality is reduced _______ and poor ______ with reduced _______ that results from impaired ______ filling of the massively hypertrophied left ventricle.

In addition, there may be dynamic obstruction to the ____________, as explained earlier.

A

chamber size ; compliance

stroke volume

diastolic

left ventricular outflow

66
Q

HYPERTROPHIC CARDIOMYOPATHY: CLINICAL FEATURES.

The limitation of cardiac output and a secondary increase in pulmonary venous pressure cause _________

A

exertional dyspnea

67
Q

HYPERTROPHIC CARDIOMYOPATHY

Owing to the massive hypertrophy, high left ventricular chamber pressure, and potentially abnormal intramural arteries, _________________ commonly results, even in the absence of concomitant __________, and thus ________ is frequent.

A

focal myocardial ischemia

coronary artery disease

anginal pain

68
Q

HYPERTROPHIC CARDIOMYOPATHY

The major clinical problems in HCM are atrial _____ with _____ formation and possibly embolization, _______ on the _____ valve, intractable cardiac failure, ventricular _______, and sudden death.

A

fibrillation

mural thrombus

infective endocarditis; mitral

arrhythmias

69
Q

HCM is one of the most common causes of sudden unexplained death in young athletes.

T/F

A

T

70
Q

HYPERTROPHIC CARDIOMYOPATHY

Many patients are stable over many years of observation, and some improve.

T/F

A

T

71
Q

HYPERTROPHIC CARDIOMYOPATHY

Different specific responsible gene mutations carry vastly differing clinical expression and prognosis.

T/F

A

T

72
Q

HYPERTROPHIC CARDIOMYOPATHY

the 403 ___ →____ mutation in the ___________ gene (the most frequent mutation observed) yields (mild or severe?) disease and a (low or high?) incidence of sudden death;

the substitution 908 ____→___ in this same gene is associated with a (low or high?) incidence of cardiac complications.

A

Arg; Gln; beta- myosin heavy chain; severe; high

Leu; Val; low

73
Q

HYPERTROPHIC CARDIOMYOPATHY

Pathways of dilated and hypertrophic cardiomyopathy, emphasizing several important concepts.

_____ forms of dilated cardiomyopathy and virtually ____ forms of hypertrophic cardiomyopathy are genetic in origin.

A

Some

all

74
Q

HYPERTROPHIC CARDIOMYOPATHY

Pathways of dilated and hypertrophic cardiomyopathy, emphasizing several important concepts.

The genetic causes of dilated cardiomyopathy involve mutations in any of a wide variety of proteins, predominantly of the _______, but also the _____,________, and _________

In contrast, the mutated genes that cause hypertrophic cardiomyopathy encode proteins of the _____.

A

cytoskeleton

sarcomere, mitochondria, and nuclear envelope.

sarcomere

75
Q

HYPERTROPHIC CARDIOMYOPATHY

Pathways of dilated and hypertrophic cardiomyopathy, emphasizing several important concepts.

Although these two forms of cardiomyopathy differ greatly in ___________ and _________, they share a common ________________

A

subcellular basis and morphologic phenotypes

pathway of clinical complications.

76
Q

RESTRICTIVE CARDIOMYOPATHY

Restrictive cardiomyopathy is a disorder characterized by a primary decrease in ________, resulting in impaired _______ during _______;

A

ventricular compliance

ventricular filling

diastole

77
Q

RESTRICTIVE CARDIOMYOPATHY

The contractile (systolic) function of the left ventricle is usually affected.

T/F

A

F

unaffected

78
Q

RESTRICTIVE CARDIOMYOPATHY

The functional state can be confused with that of _______________ or ________

A

constrictive pericarditis or HCM.

79
Q

Restrictive cardiomyopathy can be idiopathic

T/F

A

T

80
Q

RESTRICTIVE CARDIOMYOPATHY
Restrictive cardiomyopathy can be associated with distinct diseases that affect the myocardium, principally _____,______ ,________, metastatic tumor, or products of _________.

A

radiation fibrosis, amyloidosis, sarcoidosis

inborn errors of metabolism

81
Q

RESTRICTIVE CARDIOMYOPATHY

These can often be diagnosed by ___________________

A

endomyocardial biopsy.

82
Q

RESTRICTIVE CARDIOMYOPATHY: MORPHOLOGY.
In idiopathic restrictive cardiomyopathy,

the ventricles are of approximately _____ size or ________, the ventricular cavities are ___________, and the myocardium is (loose or firm?) .

A

normal; slightly enlarged

not usually dilated

Firm

83
Q

RESTRICTIVE CARDIOMYOPATHY : MORPHOLOGY.

_______ dilation is commonly observed.

A

Bi-atrial

84
Q

RESTRICTIVE CARDIOMYOPATHY : MORPHOLOGY.

Microscopically, there is often only ____ or ______ interstitial fibrosis, which can vary from _______ to ______

A

patchy or diffuse

minimal to extensive.

85
Q

Other restrictive conditions- Endomyocardial fibrosis

Is principally a disease of children and young adults in ______ and other _____ areas.

A

Africa; tropical

86
Q

Other restrictive conditions- Endomyocardial fibrosis

Characterized by fibrosis of the _______ and __________ that extends from the _____ toward and often involving the ________ and _________.

A

ventricular endocardium and subendocardium

apex

tricuspid and mitral valves

87
Q

Other restrictive conditions- Endomyocardial fibrosis

The fibrous tissue markedly diminishes the ______ and ______ of affected chambers and so induces a ______ functional defect.

Ventricular mural thrombi sometimes develop, and there is a suggestion that the fibrous tissue results from their ________.

A

volume and compliance

restrictive

organization

88
Q

The cause of Endomyocardial fibrosis is ???

A

The cause is unknown.

89
Q

Loeffler endomyocarditis

Is also marked by __________ typically with large mural thrombi similar to those seen in the _____ disease, but cases are not _____________

A

endomyocardial fibrosis

tropical

restricted to a specific geographic area.

90
Q

Loeffler endomyocarditis

In addition to the cardiac changes, there is often an ________ or ________.

The circulating ______ generally have measurable ______ and _______ abnormalities, and many are __________.

A

eosinophilia or eosinophilic leukemia

Eosinophils; structural and functional

degranulated

91
Q

Loeffler endomyocarditis

The release of toxic products of eosinophils, especially ________, is postulated to initiate ______ damage, with subsequent foci of _______ accompanied by an ________ infiltrate.

This damage is followed by ____ of the necrotic area, layering of the endocardium by ______, and finally _______ of the ______.

A

major basic protein

endocardial; endomyocardial necrosis

eosinophilic

scarring; thrombus; organization; thrombus

92
Q

Loeffler endomyocarditis

Eosinophilic endomyocardial disease has a (poor or good?) prognosis, but benefits are reported from ______________.

A

Poor

surgical endomyocardial stripping

93
Q

Endocardial fibroelastosis

Is a (common or rare?) heart disease of (obvious or obscure?) cause characterized by focal or diffuse __________ usually involving the _____________.

A

Rare

Obscure

fibroelastic thickening

mural left ventricular endocardium

94
Q

Endocardial fibroelastosis

Most common in the first ____ years of life and (often or rarely?) encountered in adults.

A

2

Rarely

95
Q

Endocardial fibroelastosis

It is often accompanied by some form of ______________, most often _____________ in about one third of all cases.

A

congenital cardiac anomaly

aortic valve obstruction

96
Q

Endocardial fibroelastosis

The significance of endocardial fibroelastosis depends on the ___________

A

extent of involvement.

97
Q

Endocardial fibroelastosis

When focal, it may have ____ functional importance

if diffuse, it may be responsible for (slow or rapid?) , progressive cardiac _______ and ________, particularly in (children or adults?) .

A

no

Rapid

decompensation and death

Children

98
Q

Doxo rubicon is an Aetiology for ???

Cocaine is an Aetiology for????

A

DCM

DCM

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