Cardiomyopathy

Question 1

What is the definition of cardiomyopathy?

Answer 1

heart disease resulting from a primary abnormality in the myocardium

Question 2

List two types of surgical procedures to correct HOCM.

Answer 2

Open heart and excision - Alcohol ablation done through cardiac cath

Question 3

What heart sound will likely be present with HOCM?

Answer 3

S4

Question 4

List three medications which may be used both to prevent arrhythmia and ease symptoms with HOCM.

Answer 4

Diuretics
Beta blockers
Non-dihydropyridine CCB (verapamil or diltiazem)

Question 5

Once a patient is diagnosed with HOCM what family counseling must be done?

Answer 5

Entire family must be screened for HOCM

Question 6

Angina syncope and heart failure in a child likely represents ____________.

Answer 6

HOCM (Hypertrophic Cardiomyopathy)

Question 7

Constrictive Cardiomyopathy occurs by what mechanism?

Answer 7

Pericardial sac constricts

Question 8

Restrictive Cardiomyopathy occurs by what mechanism?

Answer 8

Deposition into or between the myocardial cells

Question 9

What causes the sudden cardiac death in a HOCM patient?

Answer 9

Ventricular tachycardia that degenerates into ventricular fibrillation

Question 10

What maneuver makes the HOCM murmur louder?

Answer 10

Valsalva or standing

Question 11

What are the signs of HOCM on exam?

Answer 11

Apical lift
S4 gallop
Ejection murmur medial to apex

Question 12

What is the treatment for dilated cardiomyopathy?

Answer 12

Same as systolic heart failure

Beta blocker, Loop Diuretic, ACE inhibitor

Question 13

What are the three categories of cardiomyopathy and which is the most common?

Answer 13

Dilated (90%)
Hypertrophic
Restrictive

Question 14

What do low ejection fraction and hypertrophic (HOCM) heart have in common?

Answer 14

Sudden death due to VT

Question 15

What is the ejection fraction in a cardiomyopathy?

Answer 15

less than 40% (in the presence of increased left ventricular dimension)

Question 16

Which chamber is most affected in cardiomyopathies?

Answer 16

Left ventricle

Question 17

What chambers are affected (and how) in Dilated cardiomyopathy?

Answer 17

enlargement and dilation of all four chambers

Question 18

What is the most common cause of non-ischemic DCM in the US?

Answer 18

chronic alcoholism

Question 19

What is the second most common cause of non-ischemic cardiomyopathies? (#1 in other parts of the world)

Answer 19

viral myocarditis

Question 20

What is damaged in DCM and what does it result in?

Answer 20

myocytes are injured and necrosis occurs with myocardial fibrosis when muscle cells fail they dilate

Question 21

Does DCM have a systolic or diastolic dysfunction

Answer 21

systolic

Question 22

What is the number one specific cause of dilated cardiomyopathy overall?

Answer 22

Ischemic cardiomyopathy (more than 60% of patients with heart failure have this)

Question 23

What is seen histologically in DCM?

Answer 23

nonspecific abnormalities-variations in myocyte size\n-myocyte vacuolization-fibrosis and fat replace myocardium

Question 24

How does alcoholism lead to DCM?

Answer 24

alcohol destroys microvilli brush border and leads to nutritional deficits (thiamine in particular) (glutamine helps regenerate)
alcohol is toxic to cardiac tissue-> myocyte death

Question 25

What does the heart of someone with DCM resemble?

Answer 25

basketball

Question 26

In which chamber are changes seen in HCM?

Answer 26

LVH w/o ventricular chamber dilation

Question 27

What is HCM characterized by?

Answer 27

myocardial hypertrophy abnormal diastolic filling 1/3 of cases intermittent ventricular outflow obstruction

Question 28

Which wall of the ventricle is more hypertrophic in HCM?

Answer 28

the septum of the L ventricle is larger than the free wall

Question 29

What is seen histologically in HCM?

Answer 29

hypertrophy of myocardial fibers\n-interstitial fibrosis

Question 30

Where is the mutation that leads to HCM located?

Answer 30

any one of several genes that encode proteins within the sarcomere

Question 31

What is seen on an Echo for HCM?

Answer 31

hypertrophic septum and LV wall\n-small ejection fraction

Question 32

What happens to the myocardium in restricted cardiomyopathy?

Answer 32

The myocardium is infiltrated with abnormal tissue-> impaired ventricular wall motion and abnormal contraction and relaxation

Question 33

What are three causes of restrictive cardiomyopathy and which are the most common?

Answer 33

amyloidosis- most common\n \n-hemochromatosis-most common\niron overload(genetic)- kills cell\n \n-sarcoidosis

Question 34

What population is most susceptible to RCM?

Answer 34

men of northern European descent

Question 35

List 6 causes of restrictive cardiomyopathy

Answer 35

``` Amyloidosis Hemochromatosis Sarcoidosis Scleroderma Fibrosis Cancer ```