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DMST 367 Echo 3 > Restrictive And Other Cardiomyopathies > Flashcards

Restrictive And Other Cardiomyopathies Flashcards

1
Q

What does this image represent?

A

Dip and plateau/ square root sign

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2
Q

Why is there a different in waveform for the two?

A

Top is elevated filling pressures and the bottom is normal

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3
Q

What does this image represent? And what features are present? 4

A
  1. Amyloidosis
  2. LVH present
  3. Speckled myocardium
  4. Small pericardial effusion
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4
Q

What does this image represent? What is evident symptoms?

A
  1. Amyloidosis
  2. LVH and myocardial speckling evident
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5
Q

What is the difference between the waveforms?

A
  1. Left: Normal heart 10cm/s
  2. Amyloid - S’ = 4cm/s
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6
Q

RWMA don’t correspond to what in sarcoidosis?

A

Coronary arteries

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7
Q

What does this image represent?

A

RCM- HES

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8
Q

What does this image represent?

A

Fabry disease

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9
Q

What does this image represent?

A

ARVC/D

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10
Q

What does this image represent?

A

LV non-compaction

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11
Q

What does this image represent?

A

LV non-compaction

Look at the trabecular

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12
Q

What does this image represent?

A

Non-compaction of the LV

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13
Q

What does this image represent?

A

Non-compaction of LV

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14
Q

What is restrictive cardiomyopathy RCM?

A

A type of cardiomyopathy characterized by increased resistance to ventricular filling due to increased myocardial stiffness, decreased compliance, or both

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15
Q

What is RCM associated with? 2

A

Increased filling pressures such as:
1. Increased LVED
2. Increased LAP

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16
Q

What is the etiology of RCM?

A

Abnormal infiltration, storage or fibrosis within the myocardium

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17
Q

What are non-infiltrative restrictive CMO causes? 5

A
  1. Idiopathic CMO
  2. Familial CMO
  3. Hypertrophic CMO
  4. Scleroderma (scars in the heart)
  5. Diabetic CMO Fibrosis
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18
Q

What is causes of infiltrative RCM? 4

A
  1. Amyloidosis
  2. Sarcoidosis
  3. Hurler’s disease
  4. Loeffler’s disease
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19
Q

What is causes of storage disease RCM?5

A
  1. Endomyocardial fibroelastosis
  2. Carcinoid heart disease
  3. Radiation
  4. Chemo effects
  5. HES
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20
Q

Hypertrophic CMO is a separate class from RCM but can cause what? Therefoer it can also be considered what?

A
  1. Restrictive physiology
  2. Non-infiltrative form of RCM
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21
Q

How do we get a “dip and plateau”/ “square root” sign? 4

A
  1. Marked increase in LA Pressure
  2. Rapid rise in LV pressure with early diastolic filling
  3. Rapid equalization of LV and LA pressure
  4. Abrupt termination of early diastolic filling
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22
Q

Along with dip and plateau and square root, what is also seen with RCM filling pressures? 2

A
  1. Constrictive pericarditis
  2. Lots of early diastolic filling, but almost no late diastolic filling
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23
Q

What are some RCM clinical findings? 4

A
  1. DDfxn =HFpEF
  2. ECG
  3. CXR
  4. Ausculations
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24
Q

What is the clinical finding of HFpEF for RCM?

A

HF due to elevated LVEDP + LAP

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25
Q

What is the Clinical findings of ECG with RCM?

A

Arrhythmias such as:
1. A-fib (Common)
2. PVCs

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26
Q

What is clinical findings of CXR for RCM? 2

A
  1. Cardiomegaly
  2. Pulmonary congestion
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27
Q

What are clinical auscultations findings seen with RCM? 2

A

S3 and S4

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28
Q

What are some signs of left sided heart failure? 9

A
  1. Paroxysmal nocturnal dyspnea
  2. Elevated pulmonary wedge pressure
  3. Pulmonary congestion
  4. Restlessness
  5. Confusion
  6. Tachycardia
  7. Exertional dyspnea
  8. Fatigue
  9. Cyanosis
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29
Q

What are some right sided heart failure symptoms? 8

A
  1. Fatigue
  2. Increase in peripheral venous pressure
  3. Ascites
  4. Enlarged liver and spleen
  5. May be secondary to chronic pulmonary problems
  6. Distended jugular veins
  7. Anorexia and complications of GI distress
  8. Dependent edema
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30
Q

Most infiltrative disease affect what? Except what?

A
  1. LV
  2. ARVD
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31
Q

ARVD is typically associated with what side of the heart?

A

Right side

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32
Q

What are RCM echo findings? 4 (Anatomy)

A
  1. Bi-atrial enlargement
  2. +/- LVH
  3. Normal LV chamber size
  4. DDfxn grade 1-3
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33
Q

Why is there Bi-atrial enlargement for RCM?

A

RCM mimics constrictive pericarditis, but bi-atrial enlargement indicates RCM

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34
Q

DDFxn of RCM is dependent on what?

A
  1. Severity
  2. May be normal funciton in early stages
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35
Q

What is the role of echo in terms of assessment for RCM? 3

A

Assess:
1. Chamber size and function
2. Degree of diastolic function
3. Degree or MR or TR

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36
Q

What do we need to determine with echo for RCM? 4

A
  1. RVSP
  2. RCM vs constrictive pericarditis
  3. Degree of bi-atrial enlargement
  4. Presence of pericardial effusion
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37
Q

What are the medical treatments for RCM? 2

A
  1. CHF drugs such as diuretics and ACE inhibitors
  2. Anticoagulants (A-fib)
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38
Q

What are surgical treatments for RCM? 3

A
  1. Pacemaker
  2. Implantable cardioverter defibrillator (ICD)
  3. Cardiac transplant (poor long term results)
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39
Q

What are five restrictive CMO types?

A
  1. Amyloidosis
  2. Sarcoidosis
  3. Hemochromatosis
  4. HES
  5. Fabry disease
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40
Q

What is amyloidoses?

A

Infiltrative disease characterized by the deposition of amyloid fibrils in the extracellular space of various tissues

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41
Q

What is the most common type of RCM?

A

Amyloidosis

42
Q

With amyloidosis, there is a deposit of what between myocytes?

A

Amyloid proteins

43
Q

Why is diagnosis of amyloidosis challenging? What is it misdiagnosed as commonly?

A
  1. Requires biopsy
  2. Misdiagnosed in early stages as LVH due to HTN
44
Q

What organs may amyloidosis affect? 3

A
  1. Kidneys
  2. Liver
  3. Heart
45
Q

What are different amyloid disease types? 3

A
  1. Primary/ idiopathic
  2. Secondary
  3. Familial (hereditary)
46
Q

Primary/ idiopathic amyloid disease affects which demographic more?

47
Q

What is secondary amyloid disease associated with?

A

Other inflammatory disorders like R.A., Tuberculosis, chrohn’s disease

48
Q

What is another name for familila amyloid disease?

A

Transthyretin (TTR) type CMO

49
Q

Who is most likely affected by Familial amyloid disease?

A

Senile males

50
Q

What does the ECG look like for amyloid disease?

A

Low voltage ECG from

51
Q

Why is there low voltage ECG with amyloid disease?

A

myocardial damage

52
Q

In terms of amyloid disease, in addition to low voltage ECG, what is a very sensitive sign of amyloid?

53
Q

What does the CXR look like for amyloid?3

A
  1. Cardiomegaly
  2. Pulmonary venous congestion
  3. Pleural effusion
54
Q

What can Cardiac MRI do for Amyloid disease? 2

A
  1. Assess the extent of amyloid deposition
  2. Better tissue characterization than echo
55
Q

What are some 2D echo features of amyloidosis? 5

A
  1. Granular appearance of the myocardium
  2. Concentric LVH + RVH
  3. Mild-moderate bi-atrial enlargement
  4. Pericardial effusion
  5. Evidence of PHTN
56
Q

In terms of amyloid disease, what leads to more more severe diastolic dysfunction?

A

The greater the wall thickness

57
Q

In terms of amyloid disease, what results in the most restrictive filling pattern in most patients? (Where is it?)

A

> 15mm in the IVS/PW

58
Q

What are some quantitative values of amyloid disease?4

A
  1. Mitral E/A >2.0
  2. Decel time <160ms
  3. TDI e’ septal <5cm/s
  4. E/e’ ratio >14
59
Q

What is early changes for amyloid look like? (LV systolic function)

A

LV systolic function and size may be normal

60
Q

What does late changes of Amyloid disease look like? (LV systolic function) 4

A
  1. LV may dilate
  2. LVEF may reduce
  3. Mimics DCM as the thick LV muscle starts to thin and the LV dilates
  4. LA will dilate
61
Q

What is sarcoidosis?

A

Infiltrative, granulomatous disease affecting multiple organs, which involves the heart in 25% of patients

62
Q

Which demographic is primary affected by sarcoidosis?

A
  1. Females 2:1
  2. Young Africans
  3. Japanese
63
Q

How common is sarcoidosis?

64
Q

What does the granulomas infiltrate with sarcoidosis? 5

A
  1. Lungs
  2. Lymph nodes
  3. Liver
  4. Spleen
  5. Skin
65
Q

What are some clinical features of sarcoidosis?

A
  1. Progressive HF S/S
  2. sudden death
  3. ECG features
66
Q

What are some ECG features of sarcoidosis?

A
  1. Ventricular arrhythmias
  2. Heart block
67
Q

The Dx for sarcoidosis is confirmed with what?

A

Myocardial biopsy

68
Q

What are some early findings for sarcoidosis?

69
Q

What are some late findings for sarcoidosis? 3

A
  1. RWMA
  2. Segmental wall thinning (fibrosis)
  3. LV dilation and decreased EF
70
Q

What wall is affected by sarcoidosis in terms of thinning?

A

Basal anteroseptal wall

71
Q

What is hemochromatosis?

A

Iron storage disease characterized by the accumulation of excessive iron within the cells of various organs

72
Q

What are the two types of hemochromatosis?

A
  1. Primary
  2. Secondary
73
Q

Which gender is affected more with hemochromatosis? When does it become more apparent?

A
  1. Females
  2. Middle ages
74
Q

What is primary hemochromatosis?

A

Metabolic syndrome with excessive absorption of iron from diet and reduced elimination

75
Q

What is secondary hemochromatosis?

A

Iron overload due to repeated blood transfusions in patients with chronic anemia, prolonged hemodialysis alcoholic liver disease

76
Q

What is the hemochromatosis clinical pentad? 5

A
  1. CHG
  2. Liver cirrhosis
  3. Impotence
  4. Diabetes
  5. Arthritis
77
Q

What is seen physically with hemochromatosis?2

A
  1. High blood iron levels
  2. “Bronze” skin
78
Q

What does HES stand for?

A

Hypereosinonphilic syndrome

79
Q

What is HES?

A

Blood disorder characterized by persistently elevated blood eosinophil counts without an identifiable underlying etiology

80
Q

HES affects which side of the heart?

81
Q

What is needed to confirm diagnosis of HES?

82
Q

What is the pathophysiology of HES? 4

A
  1. Eosinophils infiltrate the endocardium
  2. Mural thrombus and scarring
  3. Thrombus is replaced by thrombosis
  4. This stage may mimic DCM or RCM
83
Q

What is Fabry disease?

A

Characterized by symmetric or asymmetric ventricular hypertrophy, conduction defects and aortic root dilation due to the accumulation of excessive lysome storage

84
Q

What are five echo features of Fabry disease?

A
  1. Hyperechoic endocardium
  2. LVH
  3. Diastolic dysfunction
  4. Bi-atrial enlargement
  5. Ao root dilatation
85
Q

Fabry disease may present as what?

A

Depending on the manifestation
1. HCM
2. DCM
3. RCM

86
Q

What are four less common infiltrative cardiomyopathies?

A
  1. ARVD
  2. LV non-compaction
  3. Carcinoid heart disease
  4. Collagen disorders
87
Q

What are two examples of collagen disorders?

A
  1. Lupus
  2. Rheumatoid artritis
88
Q

What does ARVD stand for?

A

Arrhythmogenic RV dysplasia/ cardiomyopathy

89
Q

What is ARVD?

A

A progressive replacement of the RV myocardium by fibrous and fatty tissue, resulting in wall thinning and aneurysm formation

90
Q

How common is ARVD?

91
Q

What is the etiology of ARVD?

92
Q

What are clinical signs of ARVD? 3

A
  1. Potentially asymptomatic
  2. Arrhythmias > sudden death
  3. CHF s/s
93
Q

What is the most diagnostic way to determine ARVD?

A

Cardiac MR

94
Q

What are Echo features of ARVD? 5

A
  1. RV systolic and diastolic dysfunction
  2. RV dilation, RV small aneurysms
  3. RV trabeculations get bigger
  4. Hyper-reflective moderator band
  5. Normal LV
95
Q

What is LV non-compaction?

A

A genetic cadiomyopathy characterized by prominent trabeculations and deep recesses, most commonly in the LV, which communicate with the ventricular cavity

96
Q

What sign is seen clinically with LV non-compaction?

A

Arrhythmias

97
Q

What is the best way to diagnostically see LV non-compaction? 2

A
  1. Contrast may be useful
  2. MRI better to assess extent of the disease
98
Q

LV non-compaction occurs when?

A

During embryology

99
Q

In terms of LV non-compaction, what supplies blood to the left heart?

A

Deep intertrabecular spaces from within the Left chamber, this is before coronary arteries have developed in the embryp

100
Q

In terms of LV non-compaction, Trabeculae normally do what with LV wall?

A

Compact into the LV wall starting at base and ending at apex

101
Q

Where is non-compaction seen with LV non-compaction? Why?

A

Apex, because this is the last area to compact

DMST 367 Echo 3 (27 decks)

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