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Echo 2 > C17: Restrictive and Other cardiomyopathies > Flashcards

C17: Restrictive and Other cardiomyopathies Flashcards

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1
Q

another name for restrictive cardiomyopathy (RCMO)

A

infiltrative CMO

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2
Q

define RCMO

A

referes to diseases other than ischemic heart disease which cause increased resistance to Lv filling due to increased myocardial stiffness

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3
Q

what is RCMO associated w/

A

increased LV end-diastolic pressure

increased mean LA pressure

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4
Q

can systolic fxn be preserved w/ RCMO

A

in some cases

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5
Q

main cause of RCMO

A

abnormal infiltration, storage or fibrosis w/in the myocardium

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6
Q

classifications of RCMO

A

non-infiltrative
infiltrative
storage disease
endomyocardial

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7
Q

examples of non-infiltrative causes of RCMO

A 
idiopathic
familial
hypertrophic CMO - separate class but can cause restrictive physiology so could fit here too
scleroderma
diabetic CMO (fibrosis)
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8
Q

examples of infiltrative causes of RCMO

A 
amyloidosis
sarcoidosis
hurlers disease
loefflers disease (WBCs deposited)
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9
Q

examples of storage disease causes of RCMO

A

hemochromatosis (iron storage disease)
fabry disease (fat deposits)
GSD

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10
Q

examples of endomyocardial causes of RCMO

A

endomyocardial fibroelastosis
carcinoid
radiation
chemo

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11
Q

describe infiltrative RCMO

A

disease of the myocardium secondary to a disease that produces histologic changes to the myocardium by depositing substances inside or in/b/w muscle cells… usually due to genetic or metabolic disorder

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12
Q

can infiltrative RCMO lead to restrictive filling patterns

why

A

yes

material deposited restricts LV filling by making it stiffer

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13
Q

does non-infiltrative disorders cause abnormal myocardium and increased stiffness

A

yes

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14
Q

clinical findings of RCMO

A

diastolic dysfunction and backwards heart failure:

fatigue, edema, SOBOE (due to increased LVEDP + LAP)
mod P HTN

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15
Q

RCMO can be hard to distinguish from which other pathology of the pericardium

A

constrictive pericarditis

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16
Q

in early stages of RCMO, what is systolic FXN like

later?

A

may be normal

reduced

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17
Q

which infiltrative disease doesn’t affect the LV

A

ARVD - arrhythmogenic RV dysplasia

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18
Q

RCMO 2D findings

A

usually normal LV cavity size
increased wall thickness
biatrial enlargement
sm pericardial effusion is common

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19
Q

RCMO on ECG

A

atrial and ventricular arrhythmias (afib common)
low voltage QRS
conduction defects

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20
Q

RCMO on chest Xray

A

cardiomegaly

pulmonary congestion/effusion

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21
Q

RCMO on cardiac MRI

A

may see the extent of the infiltration, better tissue characterization than echo

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22
Q

medical treatment for RCMO

A 
treat underlying etiology
diuretics
ACE inhibitors
anticoagulants
anti-arrhythmics
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23
Q

surgical treatment for RCMO

A

limited…
pacemaker/defib
heart transplant

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24
Q

most common type of infiltrative RCMO

A

amyloidosis

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25
describe amyloidosis
deposits of amyloid protein into the extracellular space b/w monocytes... produces a stiff myocardium and prevents effective filing
26
what is amyloidosis often misdiagnosed as in early stages
LVH due to HTN
27
what is needed to diagnose amyloidosis
cardiac biopsy and pathological evaluation for a definitive diagnosis....
28
what organs does amyloidosis affect
heart, liver, kidneys, spleen, skin
29
4 types of amyloidosis describe them
primary/idiopathic - more common in males secondary - associated w/ other inflammatory disorders familial (hereditary) senile - common in males over 70
30
amyloidosis on the ECG
- low voltage on ECG from myocardial damage - pseudo-infarction pattern - abnormal Q waves due to myocardial fibrosis - a fib - ventricular arrhythmias - conduction defects (LBBB)
31
what is a very important and sensitive sign for amyloidosis on 2D and ECG
LVH on 2D and low voltage on ECG
32
medical treatment for amyloidosis
digitalis, digoxin (slow HR) diuretics vasodilators (to decrease afterload) anticoagulants
33
surgical treatment for amyloidosis
``` bone marrow stem cell transplant pacemaker or defib heart transplant (poor long term results) ```
34
echo 2D features of amyloidosis
``` granular/speckled appearance of the myocardium concentric LVH and RVH wall motion abnormalities in late stages asymmetrical septal thickening mild-mod biatrial enlargement pericardial effusion evidence of P HTN ```
35
how does all thickness in amyloidosis correlate w/ Diastolic Dysfunction
thicker = more severe DD
36
IVS or LVPW thickness of what value will result in restrictive filling patterns in most patients
> 15 mm
37
w/ amyloidosis, s prime of the LV will often be what value
< 5 cm/s
38
LV systolic FX w/ early amyloidosis late?
early: often normal late: LV may dilate and EF will be reduced
39
late stages of amyloidosis are indistinguishable from which other cardiomyopathy
DCM, b/c the thick LV muscle starts to thin and the LV dilates.... LA will also dilate
40
can HCM and amyloid coexist in the same patient
yes
41
define sarcoidosis
multiple granulomatous disease which involves the heart in 25% of patients
42
sarcoidosis is more common in which gender
female
43
which organs do the granulomas infiltrate w/ sarcoidosis
``` lungs (most common) lymph nodes liver spleen skin parotid glands heart ```
44
clinical features of sarcoidosis
progressive heart failure diffuse fibrosis which leads to PHTN sudden death dysrhythmias, heart block (a lot of Av block due to infiltration of the septum)
45
which walls of the heart does sarcoidosis usually effect
basal inferolateral/lateral walls
46
how does the ECG occur w/ sarcoidosis
3rd degree AV block ventricular dysrhythmias pseudo-infarct patterns w/ prominent Q waves
47
Echo features of sarcoidosis
pericardial effusion increased anterior wall RV thickness (> 5 mm) pulmonary HTN RV dysfunction due to Pulmonary artery hypertension MR TR diastolic dysfunction
48
early US features of sarcoidosis late
early: increased wall thickness late: aneurysmal dilation of LV segmental wall thinning LV dilation and reduced EF wall motion abnormalities
49
is sarcoidosis diagnosed by echo
no
50
what is hemochromatosis
iron storage disease affecting multiple organ systems that result in organ damage and malfunction
51
what is primary hemochromatosis which gender does it affect more
genetic due to a metabolic syndrome male (usually males >40 and women >50)
52
what is secondary hemochromatosis
iron overload due to repeated blood transfusion in patients w/ chronic anemia, prolonged hemodialysis or due to chronic liver disease
53
clinical findings of hemochromatosis
``` CHF cirrhosis of the liver impotence diabetes arthritis ```
54
how does hemochromatosis appear on the ECG
AF, low voltage, SVTs, atrioventricular conduction defects (AV block)
55
echo features of hemochromatosis
features relates to CMO increased wall thickness preserved systolic function
56
another name for endocardial syndromes
hypereosinophilic
57
what is an endomyocardial syndrome causes
persistently elevated blood eosinophil counts which causes restriction due to: Idiopathic malignant reactive infectious (parasitic)
58
is it easy to see the endocardium w/ endomyocardial syndrome
yes
59
describe the evolution of endomyocardial syndrome
infiltration of eosinophils evolve to a thrombosis and cause scarring w/ risk of embolism
60
why does endomyocardial syndrome cause restriction what effect does this have
due to fibrotic tissue that lines the myocardium decrease in LV systolic function.... leading to CHF and death
61
clinical features of endomyocardial syndrome
SOB | neurological symptoms
62
what is needed to confirm the diagnosis of endomyocardial syndrome
biopsy
63
what is GSD
excessive storage of glycogen, hereditary cause
64
how does GSD affect the heart
- heart becomes enlarged and globular in shape | - thickened RV and LV and IVS w/ increased mass
65
other infiltrative cardiomyopathies
arrhythmogenic RV dysplasia carcinoid heart disease Fabrys disease collagen disorders
66
describe arrhythmogenic RV dysplasia (ARVD) how does it affect RV function and appearance
genetic condition w/ fibro-fatty infiltration of the RV systolic and diastolic dysfunction RV is dilated with larger trabeculations can cause arrhythmias doesnt affect the LV, its normal
67
another name for arrhythmogenic RV dysplasia (ARVD)
arrhythmogenic RV cardiomyopathy
68
describe LV non-compaction how does it affect function
genetic condition w/ LV wall thickening and deep grooves b/w the trabeculations hypokinetic arrhythmias and heart failure
69
which walls are affect w/ LV non-compaction
apex, mid lateral and mid inferior
70
describe the normal LV compaction process
occurs from epicardium to endocardium and base to apex... apex is last to compact
71
review chart at end of PP
/

Echo 2 (15 decks)

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