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Cardiology > Myocardial Disorders > Flashcards

Myocardial Disorders Flashcards

0
Q

There are primary and secondary causes of cardiomyopathy.

A

Yep. Just like everything else.

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1
Q

3 broad types of cardiomyopathy?

A

Dilated
Hypertrophy
Restrictive

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2
Q

What are typical causes of secondary cardiomyopathies?

A

Deposition of some substance / inflammation. (eg. amyloidosis, sarcoid, hemochromatosis, etc.)

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3
Q

3 broad types of genes that, when mutated mutated, cause cardiomyopathies?

A

Contractile-element associated.
Cytoskeleton associated.
Ion channelopathies.

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4
Q

Can different mutations in the same gene cause different types of cardiomyopathy.

A

Yep.

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5
Q

What’s the underlying problem in Arrythmogenic Right Ventricular Cardiomyopathy (ARVC)?

A

Problem with desmosomes… myocytes don’t adhere well. They’re replaced by scar and fat.
Causes dilated cardiomyopathy.

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6
Q

4 bad consequences of dilated cardiomyopathy?

A

Impaired contractility.
Mitral regurgiation.
Mural thrombi -> stroke.
Arrythmias (from scarring and dilated atria).

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7
Q

2 notable toxic causes of dilated cardiomyopathy?

A

Too much alcohol.

Adriamycin (doxorubicin) - an antineoplastic.

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8
Q

4 non-genetic causes of dilated cardiomyopathy?

A

Toxins (eg. EtOH, doxorubicin)
Prior myocarditis
Pregnancy
Hemochromatosis (does this count as non-genetic?)

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9
Q

Dilated cardiomyopathy of pregnancy. Is it good?

A

No, it’s not good. Can be quickly lethal, and women who survive should be advised not to become pregnant again.

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10
Q

If someone has hemochromatosis causing dilated cardiomyopathy, what are 2 other disorders you might expect them to have?

A

Diabetes.

Cirrhosis.

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11
Q

Does dilated cardiomyopathy usually have reduced LVEF?

A

Yes.

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12
Q

What does hypertrophic cardiomyopathy disproportionately affect in 90% of cases?

A

The interventricular septum -> Asymmetric Septal Hypertrophy (ASH)

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13
Q

What shape does ASH make the LV cavity become?

A

“banana” shaped

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14
Q

What happens in hypertrophic obstructive cardiomyopathy (HOCM)?

A

ASH leads to distortion of the anterior leaflet of the mitral valve, such that the leaflet can start to cover the LV outflow tract during systole.
Also, the bulging septum itself is an obstruction.
This happens in 1/3 (or 2/3) of cases of ASH… (given ASH, it’s common).

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15
Q

What does hypertrophic cardiomyopathy look like in histology? (3 things)

A

Disorganized, branching myofibrils.
Interstitial fibrosis.
Thickened intramyocardial arteries (poor perfusion).

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16
Q

What is possibly the underlying cause of hypertrophic cardiomyopathy?

A

Abnormal myocyte organization -> abnormal force generation.

The abnormal force generation may lead to release of growth factors.

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17
Q

How is the blood supply to the myocardium altered in hypertrophic cardiomyopathy?

A

Arteries are thicker, allowing less gas exchange. This can lead to angina and arrhythmia.

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18
Q

Can hypertrophic cardiomyopathy also cause endocarditis and mural thrombi?

A

Yes.

19
Q

Can different morphologies of hypertrophy occur? (i.e. more in apex, etc.)

A

Yeah.

20
Q

3 necessary conditions that allow LV outflow tract obstruction in HOCM?

A

Must have ASH.
Must have reduced LV cavity size.
Must have elongated anterior leaflet of the mitral valve.

21
Q

What does the LV - aortic pressure gradient look like in HOCM?

A

It’s way increased, like in aortic stenosis.

22
Q

3 treatments for the outflow tract obstruction in HOCM?

A

Myomyectomy (scoop out some septum).
Dual chamber pacing.
EtOH ablation of part of septum (get it to shrink).

But these don’t get rid of need for automatic internal defibrillator.

23
Q

When symptomatic, how might HOCM present?

A

Dypsnea (most common)
Angina
Fatigue, Pre-syncope, Syncope
Palpitations, CHF, etc. (not common)

24
Q

4 physical exam findings in HOCM?

A 
Increased S4 (atrial kick hits stiff ventricle).
Bifid, double-striking, pulse (from mid-systolic obstruction).
Systolic murmur (that decreases with increased preload).
Mitral regurgitation murmur (possibly).
25
Q

How can a HOCM murmur be distinguished from one caused by aortic stenosis?

A

HOCM murmur is increased by maneuvers that decrease preload
(Valsalva, standing).
AS murmur is increased by maneuvers increase afterload (squatting).

26
Q

Best way to prevent sudden death in hypertrophic cardiomyopathy?

A

Implanted cardioverter/defibrillator.

Genetic testing can help ID high risk pts who should get one.

27
Q

Why are ventricles stiff in restrictive cardiomyopathy (RCM)?

A

Crap is deposited in them.

Can be stuff like amyloid, or can be metastatic cancer.

28
Q

What can therapeutic radiation do to the heart?

A

Cause collagen deposition -> RCM.

29
Q

How can amyloid be seen on histology?

A

Congo red staining and polarized light -> birefringence (different colors depending on direction of polymers).
…similar to urate crystals in gout.

30
Q

3 types of amyloid most commonly found in the heart?

A

Immunoglobulin light chain -esp. from malignancy like multiple myeloma.
Soluble amyloid-associated (SAA) protein - associated with chronic inflammatory disease like RA and IBD.
Transthyretin - more in elderly.

31
Q

How is the LVEF in HCM, especially early in disease?

A

It’s normal, but CO is still low because of reduced filling.

32
Q

What is the rate of increase in LV pressure during diastolic filling vary in RCM vs. normally?

A

In RCM, pressure increases very rapidly with increases in volume (…the definition of reduced compliance).

33
Q

Why do hearts with RCM look like icecream cones?

A

Volume overload in the atria causes significant dilation.

34
Q

Problems with RCM?

A

Pulmonary and systemic venous congestion -> dypsnea, edema, etc.
Low CO -> fatigue, exercise intolerance.

35
Q

Why is giving diuretics for RCM good and bad?

A

Good because it will relieve pulmonary and systemic venous congestion. Bad because it will further reduce CO.

36
Q

Main treatment for RCM?

A

Treat the underlying cause.. if you can.

37
Q

Can digoxin be used for RCM?

A

Yes, but not when it’s due to amyloidosis.

38
Q

3 broad types of causes of myocarditis?

A

Infection of the heart.
Immune reaction.
Unknown.

39
Q

5 causes of lymphocytic myocarditis?

A 
Acute rheumatic fever.
Drug hypersensitivity (may include eosinophils).
Post-viral syndrome.
SLE etc.
Transplant rejection.
40
Q

If you see an eosinophilic myocarditis, what’s probably the cause?

A

Allergic reaction to a drug.

It also could be hypersensitivity to a parasitic infection… but that’s not common in the US.

41
Q

2 ways viruses can cause myocarditis?

A

T cells attacking virally-infected cells in the heart.

Cross-reactive T cells activated -> autoimmunity.

42
Q

What would a gross specimen from lethal myocarditis look like?

A

Dilated and flabby.

43
Q

What commonly precedes the development of infectious carditis?

A

“flu-like” symptoms… sometimes can be silent until CV symptoms start / sudden death from arrhythmia.

44
Q

Can endomyocardial biopsies be done?

A

Yes… if you think it’s myocarditis… (not sure when the benefits would outweigh the risk here. Maybe if it’s an immunocompromised patient and you want to determine fungal vs. bacterial, etc.??)

45
Q

2 varieties of myocarditis of unknown etiology with granulomas?

A

Sarcoidosis - small lesions, non-necrotic granulomas. Affects other organs.
Giant cell myocarditis - large lesions, necrotic granulomas w/ giant cells. Quite lethal. Only affects heart.

Cardiology (46 decks)

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