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Echo 2 > C17: Restrictive and Other cardiomyopathies > Flashcards

C17: Restrictive and Other cardiomyopathies Flashcards

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
Q

describe amyloidosis

A

deposits of amyloid protein into the extracellular space b/w monocytes… produces a stiff myocardium and prevents effective filing

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26
Q

what is amyloidosis often misdiagnosed as in early stages

A

LVH due to HTN

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27
Q

what is needed to diagnose amyloidosis

A

cardiac biopsy and pathological evaluation for a definitive diagnosis….

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28
Q

what organs does amyloidosis affect

A

heart, liver, kidneys, spleen, skin

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29
Q

4 types of amyloidosis

describe them

A

primary/idiopathic - more common in males

secondary - associated w/ other inflammatory disorders

familial (hereditary)

senile - common in males over 70

30
Q

amyloidosis on the ECG

A
  • 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
Q

what is a very important and sensitive sign for amyloidosis on 2D and ECG

A

LVH on 2D and low voltage on ECG

32
Q

medical treatment for amyloidosis

A

digitalis, digoxin (slow HR)
diuretics
vasodilators (to decrease afterload)
anticoagulants

33
Q

surgical treatment for amyloidosis

A 
bone marrow stem cell transplant
pacemaker or defib
heart transplant (poor long term results)
34
Q

echo 2D features of amyloidosis

A 
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
Q

how does all thickness in amyloidosis correlate w/ Diastolic Dysfunction

A

thicker = more severe DD

36
Q

IVS or LVPW thickness of what value will result in restrictive filling patterns in most patients

A

> 15 mm

37
Q

w/ amyloidosis, s prime of the LV will often be what value

A

< 5 cm/s

38
Q

LV systolic FX w/ early amyloidosis

late?

A

early: often normal
late: LV may dilate and EF will be reduced

39
Q

late stages of amyloidosis are indistinguishable from which other cardiomyopathy

A

DCM, b/c the thick LV muscle starts to thin and the LV dilates…. LA will also dilate

40
Q

can HCM and amyloid coexist in the same patient

A

yes

41
Q

define sarcoidosis

A

multiple granulomatous disease which involves the heart in 25% of patients

42
Q

sarcoidosis is more common in which gender

A

female

43
Q

which organs do the granulomas infiltrate w/ sarcoidosis

A 
lungs (most common)
lymph nodes
liver
spleen
skin
parotid glands
heart
44
Q

clinical features of sarcoidosis

A

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
Q

which walls of the heart does sarcoidosis usually effect

A

basal inferolateral/lateral walls

46
Q

how does the ECG occur w/ sarcoidosis

A

3rd degree AV block
ventricular dysrhythmias
pseudo-infarct patterns w/ prominent Q waves

47
Q

Echo features of sarcoidosis

A

pericardial effusion
increased anterior wall RV thickness (> 5 mm)
pulmonary HTN
RV dysfunction due to Pulmonary artery hypertension
MR
TR
diastolic dysfunction

48
Q

early US features of sarcoidosis

late

A

early: increased wall thickness

late: aneurysmal dilation of LV
segmental wall thinning
LV dilation and reduced EF
wall motion abnormalities

49
Q

is sarcoidosis diagnosed by echo

A

no

50
Q

what is hemochromatosis

A

iron storage disease affecting multiple organ systems that result in organ damage and malfunction

51
Q

what is primary hemochromatosis

which gender does it affect more

A

genetic due to a metabolic syndrome

male (usually males >40 and women >50)

52
Q

what is secondary hemochromatosis

A

iron overload due to repeated blood transfusion in patients w/ chronic anemia, prolonged hemodialysis or due to chronic liver disease

53
Q

clinical findings of hemochromatosis

A 
CHF
cirrhosis of the liver
impotence
diabetes
arthritis
54
Q

how does hemochromatosis appear on the ECG

A

AF, low voltage, SVTs, atrioventricular conduction defects (AV block)

55
Q

echo features of hemochromatosis

A

features relates to CMO
increased wall thickness
preserved systolic function

56
Q

another name for endocardial syndromes

A

hypereosinophilic

57
Q

what is an endomyocardial syndrome

causes

A

persistently elevated blood eosinophil counts which causes restriction due to:

Idiopathic
malignant
reactive
infectious (parasitic)

58
Q

is it easy to see the endocardium w/ endomyocardial syndrome

A

yes

59
Q

describe the evolution of endomyocardial syndrome

A

infiltration of eosinophils evolve to a thrombosis and cause scarring w/ risk of embolism

60
Q

why does endomyocardial syndrome cause restriction

what effect does this have

A

due to fibrotic tissue that lines the myocardium

decrease in LV systolic function…. leading to CHF and death

61
Q

clinical features of endomyocardial syndrome

A

SOB

neurological symptoms

62
Q

what is needed to confirm the diagnosis of endomyocardial syndrome

A

biopsy

63
Q

what is GSD

A

excessive storage of glycogen, hereditary cause

64
Q

how does GSD affect the heart

A
  • heart becomes enlarged and globular in shape

- thickened RV and LV and IVS w/ increased mass

65
Q

other infiltrative cardiomyopathies

A

arrhythmogenic RV dysplasia
carcinoid heart disease
Fabrys disease
collagen disorders

66
Q

describe arrhythmogenic RV dysplasia (ARVD)

how does it affect RV function and appearance

A

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
Q

another name for arrhythmogenic RV dysplasia (ARVD)

A

arrhythmogenic RV cardiomyopathy

68
Q

describe LV non-compaction

how does it affect function

A

genetic condition w/ LV wall thickening and deep grooves b/w the trabeculations

hypokinetic
arrhythmias
and heart failure

69
Q

which walls are affect w/ LV non-compaction

A

apex, mid lateral and mid inferior

70
Q

describe the normal LV compaction process

A

occurs from epicardium to endocardium and base to apex… apex is last to compact

71
Q

review chart at end of PP

A

/

Echo 2 (15 decks)

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