(MHD) Cardiomyopathies

Question 1

Define a cardiomyopathy

Answer 1

It is a primary abnormality of the myocardium. This means that cardiac dysfunction is not attributable to pressure or volume overload.

Question 2

What are the (3) general categories of cardimyopathy?

Answer 2

1. Dilated
2. Hypertrophic
3. Restrictive

Question 3

How common is dilated cardiomyopathy?

Answer 3

The most common type of cardiomyopathy

Question 4

What kind of dysfunction is dilated cardiomyopathy? What are its causes? (5)

Answer 4

It is a contractile (systolic) dysfunction.

It has genetic causes (vary)

and Non-genetic causes:

1. Myocarditis
2. Peri partum (in association with pregnancy)
3. Toxicity (alcohol)
4. Idiopathic (which are actually most likely genetic)

Question 5

What is the clinical manifestation of dilated cardiomyopathy? (3)

Answer 5

Progressive systolic CHF, arrhytmias and potential thrombi w/ embolic complications.

Question 6

General treatment for dilated cardimyopathy

Answer 6

Medications or heart transplant (last resort)

Question 7

Hypertrophic cardiomyopathy is also known as … (2)

Answer 7

Idiopathic hypertrophic subaortic stenosis and hypertrophic obstructive cardiomyopathy

Question 8

Describe the changes to the heart associated with hypertrophic cardiomyopathy

Answer 8

1. Septum is more enlarged then the otherfree wall, forming Banana shaped LV cavity.
2. There is abnormal diastolic filling
3. There can be left ventricular outflow obstruction
4. There can be endocarditis as a result of the wall rubing the valve during systole.
5. Myofibral dissarray

Question 9

What is the pathogenesis for hypertrophic cardiomyopathy? How much of it is caused by genetics?

Answer 9

Mutations in genes that encode proteins of the sarcomeres, leading to hypercontractility and associated hypertrophicity.

100% genetic

Question 10

What is the #1 cause of sudden death for young athletes?

Answer 10

Hypertrophic Cardiomyopathy

Question 11

Treatment of hypertrophic cardiomyopathy (2)

Answer 11

1. Medical therapy enhancing ventricular relaxation
2. Surgical excision of some of the muscle

Question 12

Describe restrictive cardiomyopathy

Answer 12

Decrease in ventricular compliance, which leads to impeded left ventricular filling during diastole. The cavity is a normal size.

Question 13

How is systolic functin effected during restrictive cardiopathy?

Answer 13

Its function is preserved.

Question 14

What are (2) major things which can cause restrictive cardiomyopathy?

Answer 14

1. Radiation fibrosis
2. Amyloidosis

Question 15

Describe amyloidosis

Answer 15

Misfolded proteins deposit and accumulate in the heart (and other tissues), causing tissue damage. Can lead to restrictive cardiomyopathy

Question 16

What are the key common features of ALL amyloid deposits, regardess of the proteins causing them? (what is their structure? describe their staining.)

Answer 16

1. B-pleated sheet configuration
2. Stain salmon pink with congo red staining and appear “apple-green” under polarized light.

Question 17

What is the most common cause of myocarditis? How does it work?

Answer 17

Viruses (particularly enteroviruses)

Bacteria, parasites and other noninfectious methods can also cause it.

Infection causes lymphocytes to infiltrate and injury/ destroy the myocytes.

Question 18

What are the noninfectious causes of Myocarditis? (3)

Answer 18

1. Immune-mediated (hypersensitivty rxns and rheumatic fever)
2. Giant cell myocarditis
3. Sarcoidosis

Question 19

Describe the clinical manifestations of myocarditis

Answer 19

They are a spectrum.

Can range from asymptomatic to CHF or sudden cardiac death

Question 20

What is a pericardial effusion? How is it treated?

Answer 20

When there is too much fluid in the pericardial sac due to blood, pus, serous, etc.

Must put a catheter to drain the fluid if there is too much

Question 21

Less than _______mL of slowly accumulating fluid may not be clinically signficant as far as pericardial effusions are concerned

Answer 21

500mL

(acute accumulation on the other hand would be an issue, even less than 500mL)

Question 22

What is the issue with rapidly filling or large pleural effusions? What can it lead to?

Answer 22

They can compress the heart to the point that it can’t function properly and can ultimately lead to cardiac tamponade.

Question 23

What is pericarditis?

Answer 23

Inflammation of the pericardium, secondary to cardiac, thoracic or systemic process.

Question 24

Describe fibrinous pericarditis. (how does the pericardium look? What can you hear?)

Answer 24

FIbers deposit on the pericardium causing a shaggy appearance. You can often hear pericardial friction rubbing as the heart beats.

Question 25

Suppurative (Fibrinopurulent) Pericarditis

Answer 25

Purulent neutrophils cover the pericardium as a result of an acute bacterial infection.

Question 26

Hemorrhagic pericarditis is associated with what diseases? (2)

Answer 26

TB or malignancy

Question 27

What is the key EKG change often associated with pericarditis?

Answer 27

Diffuse ST elevation and consistent chest pain.

Question 28

What position usually makes pericarditis feel a little better?

Answer 28

When the patient stands and leans forward some, essentially letting the heart hang off and not press on the spinal column.

Question 29

What is one of the issues associated with the healing of pericarditis? What must we do as a result?

Answer 29

It is a fibrotic process so if there was signifcant damage to the pericardium, healing can cause calcify and cause a restrictive pericarditis. THe pericardial space is obliterated and diastolic expansion is severely limited.

Must surgically strip and remove constricting pericardium

Question 30

What kinds are heart failure are usually associated with cardiac transplantation?

Answer 30

Intractable heart failure

• dilated cardiomyopathy
• ischemic cardiomyopathy

Question 31

What virus is associated with post transplant lymphoma?

Answer 31

Epstein-Barr virus (same one from mono)

Question 32

Where do you biopsy heart muscle from?

Answer 32

The septum of the right heart