Cardiomyopathy

Question 1

What is cardiomyopathy?

Answer 1

Heart muscle disease:

Primary or secondary

Question 2

Primary?

Answer 2

Confined to heart muscle

Question 3

Secondary?

Answer 3

Cardiac involvement as part of a systemic disorder

Question 4

3 major patterns

Answer 4

Dilated
Hypertrophic
Restrictive

Question 5

How is a cardiac biopsy performed?

Answer 5

Venous catheter inserted through perish vein into R heart, sample taken form septum

Question 6

What’s is indication for cardiac biopsy?

Answer 6

…

Question 7

Aetiology of CM?

Answer 7

…

Question 8

Which form of CM is most common

Answer 8

Dilated

Question 9

What dysfunction do you get from dilated CM

Answer 9

Systolic

Question 10

What is another name for dilated CM?

Answer 10

Congestive CM

Question 11

What age is most common for presentation?

Answer 11

20-50

Question 12

What is mortality rate for CM?

Answer 12

50% over 2 years from presentation

Question 13

What is the aetiology of dilated CM?

Answer 13

Genetic- au dom commonest, cytoskeletal proteins affected

Acquired- myocarditis, toxins, idiopathic, something else

Question 14

How does dilated CM present clinically?

Answer 14

Progressive heart failure
Sudden death
Af
Stroke
Secondary mitral regurge due to stretching of tissue around valve, rather than problem with valve itself (CM doesn't have

Question 15

When would a biopsy be done in dilated CM?

Answer 15

To exclude other diagnoses, e.g. absence of amyloidosis, inflammation, iron, granulomas.
It is not diagnostic

Question 16

What si the treatment for dilated CM?

Answer 16

Heart transplant

Question 17

What iis hypertrophic cardiomyopathy?

Answer 17

….

Question 18

How would the heart appear in HCM?

Answer 18

Assymetrical septal hypertrophy, bulges into lumen, looks banana like on cross section

Question 19

What is differential for HCM?

Answer 19

Amyloidosis, fairy disease, hypertensive heart disease

Question 20

What is aetiology of HCM?

Answer 20

Genetic (add to this)

Question 21

Hisptologically how does HCM appear?

Answer 21

Hypertrophy, myofibre disarray, fibrosis

Question 22

What is the clinical presentation of HCM?

Answer 22

Exertional dyspnoea
Harsh systolic ejection murmur
Angina
AF
Mural thrombus and stroke
HF
Arrhythmias
Sudden death (athlete's heart - heart gets thick)

Question 23

What is treatment for HCM?

Answer 23

B blockers
Septal resection
Focal infarction technique

Question 24

What is restrictive HCM?

Answer 24

Decreased ventricular compliance - poor diastolic filling

Question 25

How to the ventricles appear?

Answer 25

Normal size, no dilatation BUT wall feels stiff

Question 26

Aetiology

Answer 26

Look up

Question 27

How does amyloid appear on an ache?

Answer 27

Large atria, normal ventricles, v bright walls due to amyloid deposition

Question 28

Histoloi

Answer 28

Variable fibrosis

Question 29

What si the treatment of RCM?

Answer 29

Beta blockers
Treatment of secondary cause
Transplat

Question 30

What is arrhythmogenic right ventricular CM?

Answer 30

Aut rec form associated with cardiocutaneous syndrome…???

Question 31

What is the appearance of

Answer 31

Fibrosis of RV wall

RV function is abnormal, regional akinesia or duskiness and (severe) global right vent dilatation and dysfunction

Question 32

What is the histology of

Answer 32

Fatty replacement of myocytes
Scarring
Myocyte vacuolation

Question 33

Name 2 examples of unclassified CM

Answer 33

LV noncompaction CM

Stress induced CM