Lecture 6: Cardiomyopathy Flashcards

1
Q

Cardiomyopathy (Def)

A

Geterogenous group of disease of mycardium w/ mechanical/electrical dysfunction w/ ventricular hypertropy or dilation

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2
Q

Are cardiomyopathies ever genetic?

A

Yes, typically.

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3
Q

To diagnose cardiomyopathy, you must exclude…

A

Coronary, valvular, and HT diseases

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4
Q

Mechanical dysfunction can lead to…(and 4 symptoms)

A

Progressive heart failure (dyspnea, fatigue, edema, syncope)

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5
Q

Electrical dysfunction can lead to…

A

Arrhythmias and sudden death

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6
Q

Two classifications of cardiomyopathy

A

Primary (confined to heart) or secondary (part of generalized systemic disorder)

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7
Q

Morphological and functional classifications of cardiomyopathy (3)

A

Dilated (progressive dilation and systolic dysfunction); Hypertrophic (decreased compliance –> diastolic dysfunction); Restrictive (stiff mycoardium, decreased compliance –> diastolic dysfunction)

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8
Q

What is the most common cardiomyopathy? Which is associated with decreased ejection fraction?

A

Dilated; dilated again!

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9
Q

100% of which morphological cardiomyopathy is related to genetics?

A

Hypertrophic

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10
Q

Dilated cardiomyopathy is generally concomitant with…

A

Hypertrophy of ventricle

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11
Q

What do we see in the heart w/ hypertrophic cardiomyopathy? (3)

A

Thickened ventricle wall, reduced LV volume, enlarged LA

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12
Q

In all 3 morphological cardiomyopathies, what is enlarged?

A

LA

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13
Q

Describe patients with dilated cardiomyopathy (age, onset)

A

Typically 40-50 years of age, insidious onset

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14
Q

The causes of dilated cardiomyopathy are…given examples

A

Diverse! Idiopathic, familial (~30%), infectious (viral = coxsackie B), inflammatory, toxic (alcohol), metabolic, muscular dystrophy

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15
Q

What gene is associated with dilated cardiomyopathy?

A

TTN, encodes for titin, the largest protein expressed in humans

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16
Q

Describe common features of dilated heart

A

Large, globular, flabby heart; dilation of AV valves, fibrous thickening, mural thrombosis (stasis)

17
Q

Histologic features of dilate cardiomyopathy are…Name a few findings

A

Non-specific; myocytes w/ box-car nuclei, some myocytes are attenuated/irregular; interstitial and endocardial fibrosis

18
Q

What are some disease-related causes of dilated cardiomyopathy? What do we find with viral cardiomyopathy?

A

Viral: Coxsackie, adenovirus (white spots, congested vessels, lymphocyte aggregates); Parasitic: Chagas (amastigoes within cytoplasm)

19
Q

Three causes of noninfective cardiomyopathy

A

Granulomatous inflammatory disease; Hypersensitivity myocarditis; Collagen vascular disease

20
Q

What is diagnostic of granulomatous inflammatory diseae?

A

Multinucleated giant cells

21
Q

What do we see with sarcoidosis?

A

White patchy discoloration and non caseating granulomas

22
Q

What do we see with hypersensitivity myocarditis?

A

Eosinophil-rich infiltrate

23
Q

What drugs/toxins can lead to dilated cardiomyopathy?

A

Alcohol, cocaine, chemotherapeutic agents

24
Q

T/F: Alcohol is a major cause of dilated cardiomyopathy

25
What is a cause of dilated cardiomyopathy that only women get?
Peripartum
26
What is a metabolic cause of dilated cardiomyopathy?
Hemochromatosis (iron storage disorder)
27
Hypertrophic cardiomyopathy is an important cause of sudden cardiac death in...
Young males
28
What is the most common gene related to hypertrophic cardiomyopathy? What kind of heredity?
Genes encoding beta-myosin heavy chain; AD w/ complete penetrance
29
The hypertrophy is...
Asymmetric: proportionately greater hypertrophy of the interventricular septum (IVS) than the free LV wall
30
What causes fatal arrhythmias in hypertrophic cardiomyopathy?
Aberrant myofibers in conduction system
31
How can you tell HT and HCM apart grossly?
HT = concentric, symmetric; HCM = asymetric septal hypertrophy
32
What is a characteristic gross feature of hypertrophic cardiomyopathy in the mitral valve?
White-ish mitral valve impact lesion
33
Histologically, what do we see in hypertrophic cardiomyopathy? Why is this important?
Myocardial fiber disarray; regular conduction is not possible --> arrhythmia
34
Restrictive cardiomyopathy is the ________ common
Least
35
Primary and secondary etiology of restrictive...
Primary: tropical endomyocardial fibrosis; Secondary: amyloidosis (most common), sarcoidosis, storage/metabolic disorders (hemochromatosis)
36
Describe Loefflers endomyocarditis
Males, temperate climates, death w/in months, hypereosinophilic states
37
What do we see in amyloidosis? What stains? What is most common?
Gross: englarged atrium w/ waxy appearance; histologically: pale pink inflitrate; CongoRed (apple green) and immunoflourescent tests; AL
38
What is Arhythmogenic Right Ventricular Cardiomyopathy (ARVC)? What happens grossly?
Rare, inherited disease with mutations in desmosomal proteins that primarily affects RIGHT ventricle; fatty tissue replaces heart muscle
39
What is Left Ventricular Noncompaction (LVNC)? What happens grossly?
Arrest in endomycoardial morphogenesis in embryonic development; sponge-like appearance of left ventricular wall