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

A

True

25
Q

What is a cause of dilated cardiomyopathy that only women get?

A

Peripartum

26
Q

What is a metabolic cause of dilated cardiomyopathy?

A

Hemochromatosis (iron storage disorder)

27
Q

Hypertrophic cardiomyopathy is an important cause of sudden cardiac death in…

A

Young males

28
Q

What is the most common gene related to hypertrophic cardiomyopathy? What kind of heredity?

A

Genes encoding beta-myosin heavy chain; AD w/ complete penetrance

29
Q

The hypertrophy is…

A

Asymmetric: proportionately greater hypertrophy of the interventricular septum (IVS) than the free LV wall

30
Q

What causes fatal arrhythmias in hypertrophic cardiomyopathy?

A

Aberrant myofibers in conduction system

31
Q

How can you tell HT and HCM apart grossly?

A

HT = concentric, symmetric; HCM = asymetric septal hypertrophy

32
Q

What is a characteristic gross feature of hypertrophic cardiomyopathy in the mitral valve?

A

White-ish mitral valve impact lesion

33
Q

Histologically, what do we see in hypertrophic cardiomyopathy? Why is this important?

A

Myocardial fiber disarray; regular conduction is not possible –> arrhythmia

34
Q

Restrictive cardiomyopathy is the ________ common

A

Least

35
Q

Primary and secondary etiology of restrictive…

A

Primary: tropical endomyocardial fibrosis; Secondary: amyloidosis (most common), sarcoidosis, storage/metabolic disorders (hemochromatosis)

36
Q

Describe Loefflers endomyocarditis

A

Males, temperate climates, death w/in months, hypereosinophilic states

37
Q

What do we see in amyloidosis? What stains? What is most common?

A

Gross: englarged atrium w/ waxy appearance; histologically: pale pink inflitrate; CongoRed (apple green) and immunoflourescent tests; AL

38
Q

What is Arhythmogenic Right Ventricular Cardiomyopathy (ARVC)? What happens grossly?

A

Rare, inherited disease with mutations in desmosomal proteins that primarily affects RIGHT ventricle; fatty tissue replaces heart muscle

39
Q

What is Left Ventricular Noncompaction (LVNC)? What happens grossly?

A

Arrest in endomycoardial morphogenesis in embryonic development; sponge-like appearance of left ventricular wall