Restrictive And Other Cardiomyopathies Flashcards

1
Q

Restrictive cardiomyopathy is also known as what?

A

Infiltrative CMO

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2
Q

What does RCMO refer to?

A

Increased resistance to LV filling due to increased myocardial stiffness

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3
Q

What is the main cause of RCMO?

A

Abnormal infiltration, storage or fibrosis within the myocardium

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4
Q

What is the cause of non-infiltrative RCMO?

A

Idiopathic, familial, hypertrophic (can cause restrictive physiology), scleroderma, diabetic (fibrosis)

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5
Q

What is the cause of infiltrative RCMO?

A

Amyloidosis, sarcoidosis, Hurler’s disease, Loeffler’s disease

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6
Q

What is the cause of storage disease RCMO?

A

Hemochromatosis, Fabry disease, glycogen storage disease

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7
Q

What is the cause of endomyocardial RCMO?

A

Endomyocardial fibrosis, carcinoid heart disease, radiation, toxic effects from anthracycline (chemo)

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8
Q

What is the etiology of infiltrative diseases related to RCMO?

A

Disease of heart muscle that is secondary to disease/disroder that produces histologic changes to cardiac muscle

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9
Q

What kind of diastolic function is seen with RCMO?

A

HFnEF (backwards failure)

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10
Q

What symptoms are seen with Left CHF?

A

Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, fatigue, cough, weight gain

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11
Q

What symptoms are seen with right CHF?

A

Jugular venous distension, hepatomegaly, peripheral edema, ascites, anasarca

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12
Q

What echocardiographic findings are seen with RCMO?

A

Normal LV cavity, increased wall thickness, bi-atrial enlargement, small pericardial effusion

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13
Q

What things will you see on an ECG with RCMO?

A

Atrial and ventricular arrhythmias, low voltage QRS, conduction defects

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14
Q

What things will you see on a chest xray with RCMO?

A

Cardimegaly, pulmonary congestion/effusion

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15
Q

What things will you see on a cardiac MRI with RCMO?

A

May demonstrate infiltration

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16
Q

What are medical tx for RCMO?

A

Treat underlying etiology, diuretics, ACE inhibitors, anticoagulants, anti-arrhytmics, pacemaker

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17
Q

What are surgical tx for RCMO?

A

Very limited, cardiac transplant

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18
Q

What is the most common type of RCMO?

A

Infiltrative CMO - amyloidosis

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19
Q

Why is infiltrative CMO amyloidosis misdiagnosed in early stages as LVH?

A

Due to hypertension

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20
Q

What does infiltrative CMO amyloidosis do?

A

Produces a stiff myocardium which prevents filling

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21
Q

What are the four classifications of amyloid disease?

A

Primary/idiopathic, secondary, familial, senile

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22
Q

What is secondary amyloid disease associated with?

A

Inflammatory disorders like rheumatoid arthritis, tuberculosis, Chron’s disease

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23
Q

What is senile amyloid disease also known as?

A

Transthyretin (TTR) type CMO

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24
Q

What is a very important finding for amyloid?

A

LVH on 2D plus low voltage ECG from myocardium

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25
Q

Pseudo-infarction pattern with amyloid shows as what on the ECG and is caused by what?

A

Abnormal Q waves and is caused by myocardial fibrosis

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26
Q

How does the myocardium appear with amyloid?

A

Granular (speckled)

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27
Q

What are echo features of amyloid?

A
  • Concentric LVH and RVH
  • Wall motion abnormalities
  • Asymmetrical septal thickening
  • Mild-moderate bi-atrial enlargement
  • Pericardial effusion
  • Evidence of pulmonary hypertension
28
Q

The greater the wall thickness the more severe the what?

A

The more severe the diastolic dysfunction

29
Q

A measure of >15 mm in wall thickness suggests what for patients?

A

Restrictive filling pattern

30
Q

What parameters suggest increased filling pressures?

A

Mitral E/A: >2.0
Decel time: >150 ms
TDI e’ septal: <5 cm/s
E/e’ Ratio: >15

31
Q

What late changes with amyloid may make RCMO indistinguishable from DCMO?

A

LV dilation, LVEF reduced, LA enlargement

32
Q

What is sarcoidosis?

A

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

33
Q

With sarcoidosis, granulomas infiltrate what?

A

Lungs, lymph nodes, liver, spleen, skin, parotid glands, heart

34
Q

What are the echo features of sarcoidosis?

A
  • Pericardial effusion
  • Increased anterior RV thickness >5 mm
  • Pulonary HPTN
  • RV dsfx due to PAH
  • MR/TR
  • Diastolic dysx
35
Q

What are echo features of early sarcoidosis?

A

Increased wall thickness

36
Q

What are echo features of late sarcoidosis?

A
  • Aneurysmal dilation of the LV
  • Segmental wall thinning
  • LV dilation and reduced EF
  • Wall motion abnormalities
37
Q

What is hemochromatosis?

A

Iron storage disease affecting multiple organ system that results in organ dmage and malfunction

38
Q

What is primary hemochromatosis?

A

Inherited (metabolic syndrome: excessive absorption of iron from diet and reduced elimination)

39
Q

What is secondary hemochromatosis?

A

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

40
Q

What is the clinical pentad of hemochromatosis?

A
  1. CHF
  2. Cirrhosis
  3. Impotence
  4. Diabetes
  5. Arthritis
41
Q

How does ECG appear with hemochromatosis?

A

Afib, low voltage, SVTs, atrioventricular conduction defects

42
Q

Describe primary hemochromatosis?

A

Genetic, male to female 3:1, affects men >40, women >50

43
Q

Describe secondary hemochromatosis?

A

Chronic liver disease, multiple blood transfusions

44
Q

What are the echo features of hemochromatosis?

A

Features related to dilated CMO, increased wall thickness (RVH, LVH), preserved systolic function

45
Q

What are endomyocardial syndromes also known as?

A

Hypereosinophilic syndromes

46
Q

What do endomyocardial syndromes evolve from?

A

Infiltration of eosinophils to thrombosis and scarring, with risk of embolism

47
Q

Endomyocardial syndromes refer to persistently elevated blood eosinophil counts due to:

A
  1. Idiopathic
  2. Malignant
  3. Reactive
  4. Infectious
48
Q

What is restriction due to with endomyocardial syndromes?

A

Fibrotic tissue that lines the myocardium (decreased LV function leads to CHF, and then death)

49
Q

What is ARVD?

A

Arrhythmogenic RV cardiomyopathy

50
Q

Carcinoid heart disease not only affects valves of the right heart but also what?

A

Right heart endocardium

51
Q

What is LV non-compaction?

A

Genetic LV wall thickening with deep recesses between trabeculae

52
Q

How does the compaction process normally occur?

A

Epicardium to endocardium and base to apex

53
Q

Where is the last part of the endocardium to compact?

A

Apical (where non-compaction is seen)

54
Q

How will EF appear with dilated cardiomyopathy?

A

Mod/sev decreased

55
Q

How will EF appear with hypertrophic cardiomyopathy?

A

Normal

56
Q

How will EF appear with restrictive cardiomyopathy?

A

Normal

57
Q

How will LV diastolic fx appear with dilated cardiomyopathy?

A

Abnormal

58
Q

How will LV diastolic fx appear with hypertrophic cardiomyopathy?

A

Abnormal

59
Q

How will LV diastolic fx appear with restrictive cardiomyopathy?

A

Abnormal

60
Q

How will LV mass appear with dilated cardiomyopathy?

A

Increased (due to dilation)

61
Q

How will LV mass appear with hypertrophic cardiomyopathy?

A

Asymmetric LVH variable

62
Q

How will LV mass appear with restrictive cardiomyopathy?

A

Concentric LVH

63
Q

Which chambers will dilate with dilated cardiomyopathy?

A

All 4

64
Q

Which chambers will dilate with hypertrophic cardiomyopathy?

A

LA and RA if MR

65
Q

Which chambers will dilate with restrictive cardiomyopathy?

A

LA and RA