Cardiomyopathies

Question 1

what is a cardiomyopathy?

Answer 1

a disorder within the cardiac myocytes themselves which results in abnormal cellular and hence cardiac performance

typically leads to irreversible decline in cardiac function

Question 2

T/F: in cardiomyopathies, LVEF is the most powerful risk stratifer

Answer 2

TRUE

Question 3

cardiomyopathies can have primary and secondary causes, describe each

Answer 3

1. primary cause → pathologic processes intrinsic to cardiac myocytes themselves
2. secondary cause → result of a pathologic change in cardiac myocytes brought on by a systemic disease process

Question 4

List the types of cardiomyopathies

Answer 4

1. Dilated cardiomyopathy
2. Hypertrophic cardiomyopathy
3. Restrictive cardiomyopathy

Question 5

what is the most common form of cardiac myopathy?

Answer 5

dilated cardiomyopathy

Question 6

describe dilated cardiac myopathy

Answer 6

increased heart weight, 1 or more chambers are dilated and walls are thinned

impaired systolic function with cardiac enlargement

fibrosis is common

Question 7

T/F: in dilated cardiac myopathy, there are hypertrophied myocytes with mitochondrial abnormalities

Answer 7

TRUE

thinner walls = fewer myocytes available to do work and the available myocytes have to work harder

Question 8

List causes of dilated cardiomyopathy

Answer 8

1. ETOH/toxic substances → most common cause
2. poor nutrition (B1 deficiency)
3. Idiopathic, family history
4. AIDS
5. CT
6. cancer therapies

Question 9

what are symptoms of dilated cardiomyopathy?

Answer 9

1. fatigue
2. exertional dyspnea, SOB, cough
3. orthopnea, paroxsymal nocturnal dyspnea
4. increasing edema, weight, or abdominal girth

Question 10

list signs of dilated cardiomyopathy?

Answer 10

1. Tachypnea: increased RR
2. Tachycardia
3. HTN or hypotension
4. Characteristic heart circular shape, almost like a bowling ball

Question 11

List other pertinent findings for dilated cardiomyopathy

Answer 11

1. signs of hypoxia (cyanosis, nail clubbing)
2. jugular venous distension (JVD)
3. pulmonary edema (crackles and/or wheezes)
4. enlarged liver
5. ascities or peripheral edema

Question 12

Describe nonpharmacologic management of dilated cardiomyopathy

Answer 12

1. sodium diet restricted to 2 g/day
2. fluid restriction

Question 13

describe hypertrophic cardiomyopathy

Answer 13

charactertized by a thick LV wall with a nondilated cavity

the resulting cardiac hypertrophy is out of proportion to the hemodynamic load

9 gene deficits which cause defects in sarcomeric proteins

Question 14

describe the pathology/progression of hypertrophic cardiomyopathy

Answer 14

1. genetic defect in myocytes
2. normal BP percieved as excessive by defective myocytes
3. hypertrophy occurs as a compensatory mechanism
4. ultimately heart decompensate

Question 15

T/F: hypertrophic cardiomyopathy is the single most common cause of death in apparently young people

Answer 15

TRUE

first clinical manifestation is often sudden death

Question 16

describe restrictive/infiltrative cardiomyopathy

Answer 16

characterized by restricted diastolic filling/loss of compliance and idiopathic fibrosis

systolic function is normal but EDVs are diminished because chambers cannot expand

Question 17

what are ESVs and EFs like in restrictive/infiltrative cardiomyopathy?

Answer 17

normal

Question 18

what are the symptoms of restrictive/infiltrative cardiomyopathy?

Answer 18

1. exertional dyspnea
2. abdominal swelling
3. ankle edema
4. fatigue

Question 19

List some causes of restrictive/infiltrative cardiomyopathy

Answer 19

1. scleroderma
2. diabetes
3. sarcoidosis (fibrotic scaring secondary to myocardial infiltrates)
4. hemochromatosis (excessive deposition of Fe)
5. metastatic cancers
6. secondary to anthracycline trx
7. radiation (mediastinal)

Question 20

List other cardiac pathologies

Answer 20

1. Valvular disease
2. Arrthymias
3. Pericardial disease
4. Pericarditis
5. Myocarditis
6. Aneursym
7. Diabetic heart disease

Question 21

list 2 types of valvular diseases in the heart

Answer 21

1. stenosis
2. regurgitation

Question 22

describe the valvular disease, regurgitation

Answer 22

valve leaflets fail to completely close or the edges do not fully meet. Permits backward flow of blood. Functional and anatomic implications

Question 23

describe the valvular disease stenosis

Answer 23

the leaflets do not provide a full opening for blood to flow through.

Question 24

what is an arrthymia?

Answer 24

a disturbance of rate and/or rhythm

Question 25

describe the pericardial disease, pericardial effusion

Answer 25

increased fluid in the pericardial cavity will result in the heart having to work much harder against that pressure which reduces the amount the heart can fill and pump blood effectively

this is a medical emergency

Question 26

define pericarditis

Answer 26

a swelling or irritation of the thin saclike membrane surrounding the heart

may be caused by a viral infection or heart attack. In many cases it is idiopathic

Question 27

list symptoms of pericarditis

Answer 27

1. sharp chest pain
2. pain worsened by lying supine, inhaling deeply or cough
3. leaning forward decreases pain

Question 28

what is myocarditis?

Answer 28

inflammation of myocardium

usually caused by a viral infection

can lead to HF, arrhytmia, sudden death

Question 29

what is an aneursym?

Answer 29

an abnormal stretching (dilation) in the wall of an artery, vein, or the heart with a diameter that is at least 50% greater than normal

named according to the specific site of formation

Question 30

T/F: an aneurysm is an area of weakness that can rupture?

Answer 30

TRUE

Question 31

what is a false aneurysm?

Answer 31

the wall ruptures and a blood clot is retained in an outpouching of tissue

Question 32

what are 4 things that can lead to diabetic heart disease?

Answer 32

1. Metabolic effects due to FFA, insulin resistance
2. Structural: myocardial fibrosis and ECM changes
3. Reduced perfusion due to small vessel disease
4. Autonomic dysfunction reduced HRR