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.
2
Q
In many cases, cardiomyopathies are idiopathic (i.e., of unknown cause)
T/F
A
T
3
Q
well-defined myocardial diseases may, in the end, resemble those without known causes, both functionally and structurally.
T/F
A
T
4
Q
Major advance in our understanding has given _____ of many myocardial diseases, previously considered idiopathic.
A
genetic basis
5
Q
etiologic distinctions of cardiomyopathies have become somewhat blurred
T/F
A
T
6
Q
Three clinical, functional, and pathologic patterns :
______ cardiomyopathy (______)
_______ cardiomyopathy (____)
________ cardiomyopathy
.
A
Dilated ; DCM
Hypertrophic; HCM
Restrictive
7
Q
commonest (___ %) cardiomyopathy is ?????
________ cardiomyopathy- least prevalent.
A
90
Dilated cardiomyopathy (DCM)
Restrictive
8
Q
_________ are used widely in the diagnosis of cardiomyopathies
A
Endomyocardial biopsies
9
Q
Dilated cardiomyopathy (DCM):
Left ventricular ejection fraction: ___
Mechanisms of heart failure:____
A
Less than 40%
Impairment of contraction( systolic dysfunction)
10
Q
Hypertrophic cardiomyopathy (HCM):
Left ventricular ejection fraction: ___
Mechanisms of heart failure:____
A
50-80%
Impairment of compliance ( diastolic dysfunction)
11
Q
Restrictive cardiomyopathy:
Left ventricular ejection fraction: ___
Mechanisms of heart failure:____
A
45-90%
Impairment of compliance ( diastolic dysfunction)
12
Q
DILATED CARDIOMYOPATHY
DCM is applied to a form of cardiomyopathy characterized by progressive cardiac ______,______, and _______ (______) dysfunction.
A
hypertrophy, dilation, and contractile
systolic
13
Q
DILATED CARDIOMYOPATHY
It is sometimes called ______ cardiomyopathy.
A
congestive
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
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
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
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
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
19
Q
DILATED CARDIOMYOPATHY
Genetic : Genetic influences have been documented in some cases, particularly when _____________________
A
multiple members of a family are affected.
20
Q
DILATED CARDIOMYOPATHY
DCM has a familial occurrence in ___ to ____ % of cases.
A
20 to 30
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
22
Q
In DCM, the heart is usually not heavy
T/F
A
F
It’s heavy
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
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
25
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.
free of significant narrowing
Insufficient
26
In DILATED CARDIOMYOPATHY
The coronary arteries are usually free of significant narrowing
T/F
T
27
DILATED CARDIOMYOPATHY: The histologic abnormalities in idiopathic DCM are :
-nonspecific
- usually reflect a specific causative agent.
T/F
T
F(usually do not reflect a specific causative agent.)
28
DILATED CARDIOMYOPATHY: The histologic abnormalities in idiopathic DCM
•Their severity reflects the degree of dysfunction
•the severity indicates the patient's prognosis.
T/F
F
F
Their severity does not necessarily reflect the degree of dysfunction or the patient's prognosis.
29
DILATED CARDIOMYOPATHY: The histologic abnormalities in idiopathic DCM
Most muscle cells are ______ with ______ nuclei, but many are _______ or _______
hypertrophied
enlarged
attenuated or stretched.
30
In DILATED CARDIOMYOPATHY
Interstitial and endocardial fibrosis of variable degree is present
Large subendocardial scars replace individual cells or groups of cells
T/F
T
F(small)
31
In DILATED CARDIOMYOPATHY
small subendocardial scars replace individual cells or groups of cells, probably reflecting ______________________ caused by ______- induced imbalance between ______ and ———
healing of previous secondary myocyte ischemic necrosis
hypertrophy
perfusion supply and demand.
32
DILATED CARDIOMYOPATHY
Clinical Features.
DCM may occur at any age
DCM Does not occur in childhood
T/F
T
F( it does)
33
DILATED CARDIOMYOPATHY
Clinical Features.
It presents with (slowly or rapidly?) progressive _________, but patients may slip precipitously from a _______ to _________ state.
Slowly
congestive heart failure
compensated to a decompensated functional
34
DILATED CARDIOMYOPATHY
Clinical Features.
In the end stage, patients often have ejection fractions of (more or less ?) than ___% .
Less
25
35
DCM commonly affects those ___-____ years old.
20 to 60
36
normal left ventricular ejection fraction is approximately ___ to ____%
50 to 65
37
DILATED CARDIOMYOPATHY
Fifty percent of patients die within ________, and only ___% survive longer than ___ years.
Death is usually attributable to progressive _______ or ______
2 years
25; 5
cardiac failure or arrhythmia.
38
Embolism from dislodgment of an intracardiac thrombus may occur in DCM
T/F
T
39
DILATED CARDIOMYOPATHY
___________ is frequently recommended as a fix
Cardiac transplantation
40
A variant of ___________________ is arrhythmogenic right ventricular dysplasia
DCM
41
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 ____________
right
Left
heart failure; rhythm
ventricular tachycardia.
42
In Arrhythmogenic Right Ventricular
Cardiomyopathy , Sudden death may occur.
T/F
T
43
In Arrhythmogenic Right Ventricular
Cardiomyopathy
Morphologically the (left or right?) ventricular wall is severely ______ because of ________, with extensive ____ infiltration and interstitial ______.
Right
thinned; loss of myocytes
fatty; fibrosis
44
In Arrhythmogenic Right Ventricular
Cardiomyopathy
Although a gene defect was localized on chromosome _____, the pathogenesis remains _____.
14
obscure
45
HCM is also known by such terms as ______________ and ______________ cardiomyopathy.
idiopathic hypertrophic subaortic stenosis
hypertrophic obstructive
46
HCM
It is characterized by ________, abnormal __________, and, in about one third of cases, intermittent _____________
myocardial hypertrophy
diastolic filling
left ventricular outflow obstruction.
47
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.
Heavy; Muscular ; hypo
Flabby; hypo
48
HCM is primarily a (systolic or diastolic?) rather than (systolic or diastolic?) disorder.
Diastolic
Systolic
49
HCM morphology
The essential feature is massive ______ without __________
myocardial hypertrophy
ventricular dilation.
50
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 _________________.
disproportionate
ventricular septum
asymmetric septal hypertrophy
51
HCM MORPHOLOGY
In about ____% of cases, however, the hypertrophy is symmetrical.
10
52
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 ______.
round-to-ovoid
banana-like
ventricular septum
lumen
53
HYPERTROPHIC CARDIOMYOPATHY
Although disproportionate hypertrophy can involve the entire septum, it is usually most prominent in the ______
subaortic region.
54
HYPERTROPHIC CARDIOMYOPATHY
Also often present are _____ thickening or _____ formation in the _________ and thickening of the ________ leaflet;
endocardial
mural plaque
left ventricular outflow tract
anterior mitral
55
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 _________.
anterior mitral leaflet
septum
ventricular systole
left ventricular outflow tract
midsystole
56
HYPERTROPHIC CARDIOMYOPATHY
____-stage ventricular _____ may occur (early or late?) in a (minority or majority ?) of patients.
End; dilation
Late
Minority
57
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)
hypertrophy
40; 15
58
HYPERTROPHIC CARDIOMYOPATHY: The most important histologic features of the myocardium in HCM are
(2) haphazard disarray of ____________,__________, and ____________ in sarcomeres within cells ( myofiber disarray)
bundles of myocytes, individual myocytes, and contractile elements
59
HYPERTROPHIC CARDIOMYOPATHY: The most important histologic features of the myocardium in HCM are
(3) interstitial and replacement ______.
fibrosis
60
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.
•
amyloidosis
hypertensive
subaortic septal hypertrophy.
valvular or congenital subvalvular aortic stenosis
61
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 ______.
familial; autosomal dominant
sporadic
autosomal recessive
gene penetrance
62
HCM
The genetic defects in HCM comprise mutations in any one of four genes that encode proteins of the cardiac contractile elements, the _________:
_________
_______
_________
__________
sarcomeres
beta-myosin heavy chain
cardiac troponin T
alpha-tropomyosin
myosin-binding protein C.
63
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
beta-myosin heavy chain
64
HCM
More than ____ distinct mutations in these proteins have been identified in HCM, and the molecular defect responsible for unrelated cases varies greatly.
50
65
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.
chamber size ; compliance
stroke volume
diastolic
left ventricular outflow
66
HYPERTROPHIC CARDIOMYOPATHY: CLINICAL FEATURES.
The limitation of cardiac output and a secondary increase in pulmonary venous pressure cause _________
exertional dyspnea
67
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.
focal myocardial ischemia
coronary artery disease
anginal pain
68
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.
fibrillation
mural thrombus
infective endocarditis; mitral
arrhythmias
69
HCM is one of the most common causes of sudden unexplained death in young athletes.
T/F
T
70
HYPERTROPHIC CARDIOMYOPATHY
Many patients are stable over many years of observation, and some improve.
T/F
T
71
HYPERTROPHIC CARDIOMYOPATHY
Different specific responsible gene mutations carry vastly differing clinical expression and prognosis.
T/F
T
72
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.
Arg; Gln; beta- myosin heavy chain; severe; high
Leu; Val; low
73
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.
Some
all
74
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 _____.
cytoskeleton
sarcomere, mitochondria, and nuclear envelope.
sarcomere
75
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 ________________
subcellular basis and morphologic phenotypes
pathway of clinical complications.
76
RESTRICTIVE CARDIOMYOPATHY
Restrictive cardiomyopathy is a disorder characterized by a primary decrease in ________, resulting in impaired _______ during _______;
ventricular compliance
ventricular filling
diastole
77
RESTRICTIVE CARDIOMYOPATHY
The contractile (systolic) function of the left ventricle is usually affected.
T/F
F
unaffected
78
RESTRICTIVE CARDIOMYOPATHY
The functional state can be confused with that of _______________ or ________
constrictive pericarditis or HCM.
79
Restrictive cardiomyopathy can be idiopathic
T/F
T
80
RESTRICTIVE CARDIOMYOPATHY
Restrictive cardiomyopathy can be associated with distinct diseases that affect the myocardium, principally _____,______ ,________, metastatic tumor, or products of _________.
radiation fibrosis, amyloidosis, sarcoidosis
inborn errors of metabolism
81
RESTRICTIVE CARDIOMYOPATHY
These can often be diagnosed by ___________________
endomyocardial biopsy.
82
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?) .
normal; slightly enlarged
not usually dilated
Firm
83
RESTRICTIVE CARDIOMYOPATHY : MORPHOLOGY.
_______ dilation is commonly observed.
Bi-atrial
84
RESTRICTIVE CARDIOMYOPATHY : MORPHOLOGY.
Microscopically, there is often only ____ or ______ interstitial fibrosis, which can vary from _______ to ______
patchy or diffuse
minimal to extensive.
85
Other restrictive conditions- Endomyocardial fibrosis
Is principally a disease of children and young adults in ______ and other _____ areas.
Africa; tropical
86
Other restrictive conditions- Endomyocardial fibrosis
Characterized by fibrosis of the _______ and __________ that extends from the _____ toward and often involving the ________ and _________.
ventricular endocardium and subendocardium
apex
tricuspid and mitral valves
87
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 ________.
volume and compliance
restrictive
organization
88
The cause of Endomyocardial fibrosis is ???
The cause is unknown.
89
Loeffler endomyocarditis
Is also marked by __________ typically with large mural thrombi similar to those seen in the _____ disease, but cases are not _____________
endomyocardial fibrosis
tropical
restricted to a specific geographic area.
90
Loeffler endomyocarditis
In addition to the cardiac changes, there is often an ________ or ________.
The circulating ______ generally have measurable ______ and _______ abnormalities, and many are __________.
eosinophilia or eosinophilic leukemia
Eosinophils; structural and functional
degranulated
91
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 ______.
major basic protein
endocardial; endomyocardial necrosis
eosinophilic
scarring; thrombus; organization; thrombus
92
Loeffler endomyocarditis
Eosinophilic endomyocardial disease has a (poor or good?) prognosis, but benefits are reported from ______________.
Poor
surgical endomyocardial stripping
93
Endocardial fibroelastosis
Is a (common or rare?) heart disease of (obvious or obscure?) cause characterized by focal or diffuse __________ usually involving the _____________.
Rare
Obscure
fibroelastic thickening
mural left ventricular endocardium
94
Endocardial fibroelastosis
Most common in the first ____ years of life and (often or rarely?) encountered in adults.
2
Rarely
95
Endocardial fibroelastosis
It is often accompanied by some form of ______________, most often _____________ in about one third of all cases.
congenital cardiac anomaly
aortic valve obstruction
96
Endocardial fibroelastosis
The significance of endocardial fibroelastosis depends on the ___________
extent of involvement.
97
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?) .
no
Rapid
decompensation and death
Children
98
Doxo rubicon is an Aetiology for ???
Cocaine is an Aetiology for????
DCM
DCM
