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Cardiology > Cardiomyopathies > Flashcards

Cardiomyopathies Flashcards

1
Q

What are the two classifications of cardiomyopathy?

A
  1. Primary – due to an idiopathic process effecting the myocardium
  2. Secondary – related to a specific heart muscle disease
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2
Q

Functional Classification of cardiomyopathy?
3

Anyone who passes out from exercise what cant you miss?

A
  1. Dilatated (Congestive, DCM, IDC)
  2. Hypertrophic (IHSS, HCM, HOCM)- Very high risk for vtach and sudden cardiac death
  3. Restrictive (Infiltrative) (amyloidosis, pericarditis, sarcoidosis, post bypass, radiation)

IHSS- have a hard time relaxing so prescribe beta blocker

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3
Q

Describe the dysfunction in each of the following:

  1. Dilatated (Congestive, DCM, IDC)
  2. Hypertrophic (IHSS, HCM, HOCM)
    - What does the murmur?
  3. Restrictive (Infiltrative)
A
  1. ventricular enlargement and systolic dysfunction
  2. inappropriate myocardial hypertrophy in the absence of HTN or aortic stenosis
    - aortic stenosis/flow will change thats how its different (murmur increases in intensity)
  3. abnormal filling and diastolic function
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4
Q

WHat is IDC?

WHat are two things that it affects specifically?

What do these two things lead to? 3

A

a disease of unknown etiology that principally affects the myocardium

  1. LV dilatation and
  2. systolic dysfunction
    pathology
  3. increased heart size and weight
  4. ventricular dilatation, normal wall thickness
  5. heart dysfunction out of portion to fibrosis
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5
Q

When do symtpoms occur?

How do you differentiate it from ischemia?

A

not until its very advanced disease. Cardiomegaly and CHF symptoms

The whole ventricle is affected not just an area

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6
Q

Dilated Cardiomyopathy (DCM)
-What happens to the ventricles?
-What is often the cause?
5

A
    1. Ventricles stretch and become flabby and the myocardium deteriorates
    1. Cause often unknown
      1. autoimmune
      2. Drug toxicity (alcohol, cocaine, excess catecholamines, chemotherapeutic agents),
      3. hypothyroidism
      4. inflammation of the heart

are implicated in some cases, as is just congestive heart failure

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7
Q

What is the pathophysiology behind dilated cardiomyopathy:

  1. The heart’s attempt to work harder results in?
  2. What does this activate?
  3. Because ventricular contractility is impaired what is poor?
A
  1. increasing levels of Ca2+ in the cardiac cells.
  2. This activates a calcium-sensitive enzyme initiating a cascade which switches on genes that cause heart enlargement
  3. CO is poor and the condition progressively worsens
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8
Q

Dilated Cardiomyopathy:
What two things lead to low cardiac output?

What dempgraphic is it more common in? 3

A

Systolic dysfunction and pump failure

  1. African Americans and
  2. males have 2.5x increased risk
  3. Most common age of diagnosis 20-50yrs
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9
Q

Dilated Cardiomyopathies:

  1. What funciton is impaired?
  2. Describe the ventricles?
  3. Often caused by? 3
  4. HOw will the EKG look?
  5. What does the echo show?
A
  1. Impaired systolic function : CHF
  2. Dilation of one or both ventricles
    • Often idiopathic
    • Ethanol,
    • chemotherapy
  4. Nonspecific ST –T wave changes on EKG
  5. Echo shows ventricular dilation
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10
Q

What is perpartum and what kind of cardiomyopathy is it?

A

is a form of dilated cardiomyopathy that is defined as a deterioration in cardiac function presenting typically between the last month of pregnancy and up to six months postpartum.

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11
Q

In order of most common name the etiologies most seen of dialated cardiomyopathy?
8

A
  1. IDCM
  2. Myocarditis- can usually biopsy this
  3. Ischemic CM
  4. Infiltrative disease
  5. Peripartum CM
  6. Hypertension
  7. HIV
  8. CTD (connective tissue diseases)
  9. Substance abuse
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12
Q

Most commmon diastolic dysfuncion? 2

Systolic dysfunction? 3

A
  1. Long standing hypertension
  2. Aortic stenosis
  3. Alcohol
  4. Coxaski Virus/HIV
  5. Peripatrum cardiomyopathy
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13
Q

a

A

a

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14
Q
  1. What is it hard to differentiate dilated cardiomyopathy from?
  2. When is it local dysfuntion?
  3. When is it global dysfunction?
A
  1. dilated left ventricle due to severe CAD
  2. Usually post MI/ischemia - local dysfunction
  3. Dilated myopathy - global dysfunction
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15
Q

IDIOPATHIC DILATED CARDIOMYPATHY
CLINICAL PRESENTATIONS
6

A
  1. Heart failure symptoms 75%-85%
  2. Anginal chest pain 8%-20%
  3. Emboli (systemic or pulmonary) 1%-4%
  4. Syncope
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16
Q 
Dilated Cardiomyopathy
Diagnosis
1. CXR findings? 3
2. EKG findings? 5
3. Echo findings? 3
A
  1. CXR-
    - enlarged heart!!! this should clue you in!
    - biventricular enlargement, and
    - pulmonary vascular congestion
  2. EKG-
    - LVH,
    - Left atrial enlargement,
    - Q waves,
    - poor R wave progression,
    - Afib
  3. Echo-Confirms Diagnosis!!!!!
    -ventricular enlargement,
    -increased systolic and diastolic volumes,
    -decreased EF!!!!
    LOOKING FOR CLOT
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17
Q

Management of DCM?

4

A
  1. Limit activity based on functional status
  2. Salt restriction
  3. Fluid restriction
  4. Initiate medical therapy
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18
Q

DCM medical therapy?
5

What should we consider?

A
  • ACE inhibitors, diuretics
  • digoxin
  • hydralazine / nitrate combination
  • Anticoagulants
  • Anti-arrhythmics

Consider transplant

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19
Q

Dilated Cardiomyopathy
Goals of Therapy
2

A
  1. Treat Congestive Symptoms

2. Identify Diseases that can be Treated

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20
Q

Identify Diseases that can be Treated that cause dilated cardiomyopathy?

8

A
  1. CA
  2. Thyroid Disease
  3. Hemochromatosis
  4. Sarcoidosis
  5. Infections
  6. ETOH
  7. Drugs (chemo drugs…..DOXIRUBICIN)
  8. Peripartum
21
Q
  1. What percent is Alcohol
  2. Induced Cardiomyopathy of DCM?
  3. More common in wat gender?
    Etiology? 2
  4. What do we need to remember about this?
A
  1. 3-4% of DCM
  2. Incidence more common in men
  3. Etiology
    - Direct toxic effects of ETOH
    - Malnutrition
    - –MOST LIKELY A GENETIC PREDISPOSITION TOO
  4. IT IS REVERSIBLE…………abstinence
22
Q

21

A

21

23
Q

21

A

21

24
Q
  1. What is Peripartum Cardiomyopathy?
  2. Risk factors? 5
  3. What is the cause?
  4. What so the signs and symtpoms resemble?
  5. WHy are the symptoms often ignored?
A
  1. Ventricular dysfuntion developing in the last month before delivery to 5 months postpartum
  3. Advanced maternal age
  4. African-American race
  5. Multifetal pregnancies
  6. Preeclampsia
  7. Gestational HTN
  8. Cause Uncertain
  9. Signs/Symptoms
    Resemble those of dilated cardiomyopathy
  10. Often symptoms are ignored because dyspnea, swelling and fatigue are normal symptoms at the end of pregnancy
25
Q

Diagnosis of permpartum? 3

Treatment?

Three possible outcomes?

A

Dx:
Echo, EKG, and other tests of cardiac function

Tx:
Same as for dilated cardiomyopathy

Three possible outcomes:
1/3 heart returns to normal in 6 months
1/3 cardiomegaly persists and outcome is poor
1/3 heart returns to normal on maximal medical therapy

26
Q
  1. What is Hypertrophic Cardiomyopathy characterized by?
  2. What is the usual site for hypertrophy?
  3. What do the cells look like?
  4. What are two different kinds of forms?
  5. Its often associated with what?
A
  1. Inappropriate, asymmetric myocardial hypertrophy
  2. Septum is usual site for hypertrophy
  3. Cells with bizarre appearance
  4. Familial (autosomal dominant) and sporadic forms
  5. Hypertrophic cardiomyopathy with obstruction
27
Q

Hypertrophic Cardiomyopathy:

  1. Pathological changes are of muscular hypertrophy associated with what?
  2. _________ and _________ left ventricle in the absence of another disease; small LV cavity; uncommon disease
  3. Abnormal _______ _______diastolic relaxation and filling…a diastolic disorder
A
  1. fibrosis
  2. Hypertrophied
    non-dilated
  3. compliance-impaired
28
Q

Hypertrophic Cardiomyopathy:

  1. Pathological changes are of muscular hypertrophy associated with what?
  2. _________ and _________ left ventricle in the absence of another disease; small LV cavity; uncommon disease
  3. Abnormal _______ _______diastolic relaxation and filling…a diastolic disorder
A
  1. fibrosis
  2. Hypertrophied
    non-dilated
  3. compliance-impaired
29
Q

Pathophysiology
of hypertrophic cardiomyopathy.
Three issues with what?

A
  1. Systole
  2. Diastole
  3. Mayocardial ischemia
30
Q 
Pathophysiology
of hypertrophic cardiomyopathy. 
1. Systole?
2. Diastole?
3. Mayocardial ischemia?
A
  1. Systole
    -dynamic outflow tract gradient
  2. Diastole
    -impaired diastolic filling, ↑ filling pressure
  3. Myocardial ischemia
    -↑ muscle mass, O2 demand
    systolic compression of arteries
31
Q

Hypertrophic Cardiomyopathy
Clinical Features
6

A
  1. Symptom severity progresses with age
  2. Hypertensive history
  3. Dyspnea on exertion-most common initial or presenting symptom
  4. Angina-like chest pain, palpitations and syncope may also be present
  5. Syncope
  6. Sudden death….maybe no symptoms then…..
32
Q

What should we find on PE for hypertrophic cardiomyopathy?

3

A
  1. Apex localized
  2. Palpable S4
  3. Harsh systolic ejection murmur across entire precordium → apex & heart base
33
Q

Cardiac Findings
for hypertrophic cardiomyopathy?
8

A
  1. Left ventricular hypertrophy
  2. Abnormal Q waves – deep and narrow
  3. Asymmetric septal thickening
  4. Systolic anterior motion of anterior mitral leaflet
  5. Ejection murmur LSB
  6. Often mitral regurg murmur
  7. Fourth heart sound
  8. Echocardiography: Doppler – most important
34
Q

Hypertrophic Cardiomyopathy
Diagnosis
3

A
  1. EKG-LVH in 30%,
  2. CXR-usually normal (small cavity)
  3. Echo-study of choice as usual-demonstrates hypertrophy
35
Q

Management of hypertrophic cardiomyopathy?

4

A
  1. All first degree relatives: screening…
    echocardiography/genetic counseling
  2. Avoid competitive athletic/discourage rigorous exercise
  3. Holter x 48 hours (sudden death comes from arrhythmias)
  4. Afib is the ominous predictor. heading towards vtach and fib
36
Q

Treatment
for hypertrophic cardiomyopathy?
4

A
  1. Maneuvers that ↓ end-diastolic volume
    (↓ venous return & afterload, ↑ contractility)
  2. Vasodilators (ACE_
  3. Inotropes (dig)
  4. B-blockers for longer diastolic filling time (slows HR)
37
Q

Non-responders to Medical therapy

4

A

1- Surgery (Myotomy/Myectomy) +/- MVR
2- ICD (prevents sudden death)
3- DDD pacemaker (decreases gradient)
4- NSRT (Non-surgical septal reduction therapy…..alcohol septal ablation)

38
Q

Describe 4- Alcohol septal ablation (NSRT). 2

A
  1. Controlled myocardial infarction of the basal ventricular septum
    to ↓ gradient.
  2. First septal artery occluded with a balloon catheter and ETOH injected distally
39
Q

Recommendations for Athletic Activity

2

A
  1. Avoid most competitive sports (whether or not symptoms and/or outflow gradient are present)
  2. Low-risk older patients (>30 yrs) may participate in athletic activity if all of the following are absent
40
Q

Low-risk older patients (>30 yrs) may participate in athletic activity if all of the following are absent
9

A
  1. ventricular tachycardia on
  2. Holter monitoring
    family history of sudden death due to HCM
  3. history of syncope or episode of impaired consciousness
  4. severe hemodynamic abnormalities, gradient ≥50 mmHg
  5. exercise induced hypotension
  6. moderate or severe mitral regurgitation
  7. enlarged left atrium (≥50 mm)
  8. paroxysmal atrial fibrillation
  9. abnormal myocardial perfusion
41
Q
  1. Restrictive cardiomyopathy
    is a problem of?
  2. And what makes it this?

What is it frequently accompianed by? 2

A
  1. Diastolic dysfunction
  2. the ventricular walls are excessively rigid & impede ventricular filling
  3. Frequently accompanied by small degree of depressed contractility & EF
42
Q
  1. What are the characteristics of this disease? 3

2. The atria frequently become enlarged after chronic exposure to what?

A
    • Myocardial fibrosis,
    • hypertrophy, or
    • infiltration due to variety causes…mostly idiopathic
  2. high filling pressure
43
Q

How would you describe contractility in restrictive cardiomyopathy?

So what is the underlying problem?

What will eventually happen to the atria?

A
  1. Contractility is relatively normal but
  2. because of ridigity outside (fibrotic changes) it will change filling pressure. more of a diastolic dysfuntion.
  3. eventually atria will enalrge due to chronic exposure of high pressure
44
Q
  1. Restrictive cardiomyopathy affects the myocardium how?
  2. Its a what kind of disease?
  3. the abnormal substance is largely between what?
  4. If its endomyocardial what is it due to? 2
A
  1. Deposition of abnormal substances in the myocardium
  2. Infiltrative disease:
  3. the myocytes → Amyloidosis, Sarcoidosis
  4. Endomyocardial: fibrosis or radiation
45
Q

Restrictive cardiomyopathy
clinical features?
9

A
  1. Exercise intolerance & dyspnea,
  2. dependent edema,
  3. ascites,
  4. anasarca (generalized edema)
  5. enlarged, tender, & often pulsatile liver
  6. (right sided symptoms)
  7. Thromboembolic complications: 1/3 of patients
  8. Jugular venous distension,
  9. S3, S4
46
Q 
Restrictive Cardiomyopathy
Diagnosis
1. CXR?
2. EKG?
3. ECHO?
4. Whats the best way to assess filling pressure?
5. How do we get the official diagnosis?
A
  1. CXR-signs of CHF without cardiomegaly
  2. EKG-nonspecific changes most likely
  3. ECHO-thickened LV walls with normal or slightly decreased volume
  4. whats the best way to get filling pressyre? cath
  5. official diagnosis? biopsy
47
Q
  1. Describe the muscle of the heart in amyloid heart disease?
  2. Whats the major dysfunction?
  3. Whats the ekg showing? 3
  4. What will the echo show?
  5. How could we acquire amyloid biopsy? 3
A
  1. Thickened, firm, rubbery ventricular muscle
  2. Major diastolic dysfunction
    Possible systolic dysfunction
  3. -Low voltage on EKG
    -Abnormal Q waves – look like an M.I.
    -Conduction disturbances, arrhythmias not uncommon
  4. Echo: thickened walls everywhere
  5. May find on rectal or gingival bx. May require myocardial biopsy
48
Q

Restriction (4) vs Constriction (2)

History provides important clues to distinguish between them:

A

Constrictive pericarditis

  • history of TB,
  • trauma,
  • pericarditis,
  • collagen vascular disorders

Restrictive cardiomyopathy

  • amyloidosis,
  • hemochromatosis (iron deposits)
49
Q

Treatment for Restrictive cardiomyopathy?

3

A

No satisfactory medical therapy
Drug therapy must be used with caution
-diuretics for extremely high filling pressures
-Vasodilators may decrease filling pressure
-Calcium channel blockers to improve diastolic compliance

Cardiology (19 decks)

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