Restrictive And Other Cardiomyopathies

Question 1

Restrictive cardiomyopathy is also known as what?

Answer 1

Infiltrative CMO

Question 2

What does RCMO refer to?

Answer 2

Increased resistance to LV filling due to increased myocardial stiffness

Question 3

What is the main cause of RCMO?

Answer 3

Abnormal infiltration, storage or fibrosis within the myocardium

Question 4

What is the cause of non-infiltrative RCMO?

Answer 4

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

Question 5

What is the cause of infiltrative RCMO?

Answer 5

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

Question 6

What is the cause of storage disease RCMO?

Answer 6

Hemochromatosis, Fabry disease, glycogen storage disease

Question 7

What is the cause of endomyocardial RCMO?

Answer 7

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

Question 8

What is the etiology of infiltrative diseases related to RCMO?

Answer 8

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

Question 9

What kind of diastolic function is seen with RCMO?

Answer 9

HFnEF (backwards failure)

Question 10

What symptoms are seen with Left CHF?

Answer 10

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

Question 11

What symptoms are seen with right CHF?

Answer 11

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

Question 12

What echocardiographic findings are seen with RCMO?

Answer 12

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

Question 13

What things will you see on an ECG with RCMO?

Answer 13

Atrial and ventricular arrhythmias, low voltage QRS, conduction defects

Question 14

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

Answer 14

Cardimegaly, pulmonary congestion/effusion

Question 15

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

Answer 15

May demonstrate infiltration

Question 16

What are medical tx for RCMO?

Answer 16

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

Question 17

What are surgical tx for RCMO?

Answer 17

Very limited, cardiac transplant

Question 18

What is the most common type of RCMO?

Answer 18

Infiltrative CMO - amyloidosis

Question 19

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

Answer 19

Due to hypertension

Question 20

What does infiltrative CMO amyloidosis do?

Answer 20

Produces a stiff myocardium which prevents filling

Question 21

What are the four classifications of amyloid disease?

Answer 21

Primary/idiopathic, secondary, familial, senile

Question 22

What is secondary amyloid disease associated with?

Answer 22

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

Question 23

What is senile amyloid disease also known as?

Answer 23

Transthyretin (TTR) type CMO

Question 24

What is a very important finding for amyloid?

Answer 24

LVH on 2D plus low voltage ECG from myocardium

Question 25

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

Answer 25

Abnormal Q waves and is caused by myocardial fibrosis

Question 26

How does the myocardium appear with amyloid?

Answer 26

Granular (speckled)

Question 27

What are echo features of amyloid?

Answer 27

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

Question 28

The greater the wall thickness the more severe the what?

Answer 28

The more severe the diastolic dysfunction

Question 29

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

Answer 29

Restrictive filling pattern

Question 30

What parameters suggest increased filling pressures?

Answer 30

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

Question 31

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

Answer 31

LV dilation, LVEF reduced, LA enlargement

Question 32

What is sarcoidosis?

Answer 32

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

Question 33

With sarcoidosis, granulomas infiltrate what?

Answer 33

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

Question 34

What are the echo features of sarcoidosis?

Answer 34

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

Question 35

What are echo features of early sarcoidosis?

Answer 35

Increased wall thickness

Question 36

What are echo features of late sarcoidosis?

Answer 36

- Aneurysmal dilation of the LV - Segmental wall thinning - LV dilation and reduced EF - Wall motion abnormalities

Question 37

What is hemochromatosis?

Answer 37

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

Question 38

What is primary hemochromatosis?

Answer 38

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

Question 39

What is secondary hemochromatosis?

Answer 39

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

Question 40

What is the clinical pentad of hemochromatosis?

Answer 40

1. CHF 2. Cirrhosis 3. Impotence 4. Diabetes 5. Arthritis

Question 41

How does ECG appear with hemochromatosis?

Answer 41

Afib, low voltage, SVTs, atrioventricular conduction defects

Question 42

Describe primary hemochromatosis?

Answer 42

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

Question 43

Describe secondary hemochromatosis?

Answer 43

Chronic liver disease, multiple blood transfusions

Question 44

What are the echo features of hemochromatosis?

Answer 44

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

Question 45

What are endomyocardial syndromes also known as?

Answer 45

Hypereosinophilic syndromes

Question 46

What do endomyocardial syndromes evolve from?

Answer 46

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

Question 47

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

Answer 47

1. Idiopathic 2. Malignant 3. Reactive 4. Infectious

Question 48

What is restriction due to with endomyocardial syndromes?

Answer 48

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

Question 49

What is ARVD?

Answer 49

Arrhythmogenic RV cardiomyopathy

Question 50

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

Answer 50

Right heart endocardium

Question 51

What is LV non-compaction?

Answer 51

Genetic LV wall thickening with deep recesses between trabeculae

Question 52

How does the compaction process normally occur?

Answer 52

Epicardium to endocardium and base to apex

Question 53

Where is the last part of the endocardium to compact?

Answer 53

Apical (where non-compaction is seen)

Question 54

How will EF appear with dilated cardiomyopathy?

Answer 54

Mod/sev decreased

Question 55

How will EF appear with hypertrophic cardiomyopathy?

Answer 55

Normal

Question 56

How will EF appear with restrictive cardiomyopathy?

Answer 56

Normal

Question 57

How will LV diastolic fx appear with dilated cardiomyopathy?

Answer 57

Abnormal

Question 58

How will LV diastolic fx appear with hypertrophic cardiomyopathy?

Answer 58

Abnormal

Question 59

How will LV diastolic fx appear with restrictive cardiomyopathy?

Answer 59

Abnormal

Question 60

How will LV mass appear with dilated cardiomyopathy?

Answer 60

Increased (due to dilation)

Question 61

How will LV mass appear with hypertrophic cardiomyopathy?

Answer 61

Asymmetric LVH variable

Question 62

How will LV mass appear with restrictive cardiomyopathy?

Answer 62

Concentric LVH

Question 63

Which chambers will dilate with dilated cardiomyopathy?

Answer 63

All 4

Question 64

Which chambers will dilate with hypertrophic cardiomyopathy?

Answer 64

LA and RA if MR

Question 65

Which chambers will dilate with restrictive cardiomyopathy?

Answer 65

LA and RA