Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

RC Test 4 > B4.069 Cardiomyopathies > Flashcards

B4.069 Cardiomyopathies Flashcards

(55 cards)

Start Studying
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

normal heart weight

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

RV wall thickness

A

0.3 to 0.5 cm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

LV wall thickness

A

1.3 to 1.5 cm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

hypertrophy

A

grater heart weight or ventricular thickness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

dilation

A

enlarged chamber size

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

cardiomegaly

A

increase in cardiac weight or size

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

5 classes of cardiomyopathy

A
  1. dilated (congestive)
  2. hypertrophic
  3. restrictive (infiltrative)
  4. arrhythmogenic
  5. unclassified
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

most common cardiomyopathy

A

dilated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

definition of dilated cardiomyopathy

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

pathogenesis of dilated cardiomyopathy

A 
genetic factors most common
viral myocarditis
alcohol, chemo, cobalt, cocaine, vasopressors
iron overload
immunologic abnormailities
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

how do genetic factors cause dilated cardiomyopathy?

A

defects in force transmission lead to dilatation, poor contractility

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

non specific gross pathology of cardiomyopathies

A

atrophy and hypertrophy of myocardial fibers

interstitial and perivascular fibrosis, subendocardial fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

electron microscopy findings in cardiomyopathy

A

increased mitochondria and loss of sarcomeres

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

clinical features of dilated cardiomyopathies

A 
asymptomatic LV dilatation
progressive exercise intolerance
CHF
75% die within 5 yrs
transplantation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

genetic disorders associated with hypertrophic cardiomyopathy

A

Noonan syndrome
Lentiginosis
Costello syndrome
Mitochondrial hypertrophies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

exaggerated hypertrophies (not cardiomyopathy but on differential)

Study These Flashcards
A

hypertensive LVH
athletes heart
neonatal cardiomegaly

24
Q

clinical features of hypertrophic cardiomyopathies

Study These Flashcards
A 
asymptomatic 
screening of family
sudden death
 dyspnea, angina, syncope
unsuspected HCM at autopsy of sudden death in a young competitive athlete
25
treatment of hypertrophic cardiomyopathy
B blockers Ca channel blockers surgery
26
definition of restrictive cardiomyopathies
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
2 groups of restrictive cardiomyopathies
endocardial | myocardial
28
3 conditions with endomyocardial involvement
endomyocardial fibrosis loeffler endocarditis endocardial fibroelastosis
29
endomyocardial fibrosis
equatorial Africa CHF death children and young adults
30
loeffler endocarditis
``` temperate zones CHF death men in 5th decade hypereosinophelia, myeloproliferative disorder ```
31
endocardial fibroelastosis
focal or diffuse thickening, LV | first 2 years of life
32
pathology of endomyocardial diseases
grayish white thickened endocardium mural thrombi fibrotic endocardium
33
conditions with predominant myocardial involvement
amyloidosis sarcoidosis storage disease idiopathic
34
amyloidosis pathology
cardiomegaly | amyloid infiltrate
35
clinical presentation of amyloidosis
common cause of death in patients with MM R sided heart failure arrhythmias
36
senile cardiac amyloidosis
elderly patients | transthyretin deposition-better prognosis
37
staining for cardiac amyloidosis
congo red | apple green birefringences
38
sarcoidosis
generalized granulomatous disease | non-caseating granulomas, fibrosis
39
clinical presentation of sarcoidosis
mixed pattern of dilated or restrictive cardiomegaly CHF arrhythmias sudden death
40
examples of storage diseases
``` glycogen storage diseases (pompe, cori, Anderson) mucopolysaccharidoses sphingolipidoses (fabry, gaucher) iron storage (hemochromatosis) ```
41
what happens in storage diseases
protein cannot be degraded and gets stored in muscle
42
results of hemochromatosis
Fe deposition primary or secondary CHF dilated heart, brown color, LVH, fibrosis
43
imaging of hemochromatosis
dilated brown heart | blue dots on Perl's iron stain
44
definition of arrhythmogenic cardiomyopathy
primary myocardial disease characterizes by progressive adipose of fibroadipose replacement of ventricular myocardium "RV arrhythmogenic dysplasia"
45
gross pathology of arrhythmogenic cardiomyopathy
``` cardiomegaly fatty infiltration of RV myocardium partial or transmural occasionally biventricular involvement mural thrombi ```
46
histopath findings of arrhythmogenic cardiomyopathy
fatty or fibrofatty infiltration of myocardium
47
pathogenesis of arrhythmogenic cardiomyopathy
``` congenital defect (aplasia of myocardium) genetic factors (20-30% family inheritance, mitochondrial DNA mutations) acquired factors (chronic myocarditis, immune, viral) ```
48
clinical features of arrhythmogenic cardiomyopathy
concealed form overt electrical heart disorder, typical presentation RV failure biventricular failure, late
49
myocarditis
diverse group of pathologic entities infections immune related (postviral, poststreptococcal, SLE) unknown (sarcoid, giant cell myocarditis)
50
viral myocarditis
lymph myocarditis + myocyte injury
51
Chagas disease
trypanosoma cruzi
52
hypersensitivity myocarditis
inflame infiltrate + eosinophilia
53
giant cell myocarditis
inflame infiltrate + giant cells + myocyte injury
54
clinical features of myocarditis
``` heart failure arrhythmias, dyspnea, palpitations dilated cardiomyopathy sudden death symptoms related to primary disease ```
55
specific caridomyopathies
muscular dystrophies can have cardiac manifestations neuromuscular disorders histiocytoid cardiomyopathy ischemic cardiomyopathy (common)

RC Test 4 (22 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions