8/12- Pathology of Cardiomyopathies Flashcards Preview

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Flashcards in 8/12- Pathology of Cardiomyopathies Deck (21):
1

What is another name for hypertrophic cardiomyopathy?

Asymmetric septal cardiomyopathy (?)

2

What is seen in restrictive CM in terms of space between cells (packed or spread)?

Large expansion of interstitial space

3

Causes of DCM?

Mostly idiopathic, and then genetic

- Toxin induced (alcohol, cocaine)

- Infectious

- Autoimmune

- Thyroid disorders

2ndary causes:

- Ischemic (post MI)

- Post inflammatory (after MI)

4

Describe the cardiomyopathies in terms of what you would see on microscopy?

Dilated CM:

- Collagen (interstitial fibrosis) highlighted by Masson's trichrome stain

Restrictive CM:

- interstitial deposits (e.g. amyloid deposits)

Hypertrophic CM:

- Myocyte disarray, hypertrophy and interstitial fibrosis

5

Case)

- 50 yo male with increasing fatigue, SOB for 6 wks

- Tires, SOB when walking up stairs

- Wakes at night gasping for breath

- Sleeps on 2 extra pillows

- PMH: "cold" that lasted a ong time

- SH: travel to S America for business

- Remote alcohol abuse x 15 yrs

- SOB and extra pillows is indicative of heart failure; can see pulmonary edema

6

What is the most common type of cardiomyopathy?

Dilated CM

7

What is the major dysfunction in DCM?

Systolic dysfunction and congestive heart failure

8

Gross features of DCM?

- 2-3 x heavier

- Large and flabby

- Dilation of all 4 chambers

- Ventricular thickness variable

- Mural thrombi common

9

Microscopic features of DCM?

- Variable myocyte nuclear enlargement and attenuation with irregularity

- Loss of myofibrils in myocytes

- Interstitial and subendothelial fibrosis

- Increased interstitial lymphocytes

10

Case)

- 22 yo basketball player

- Funny feeling in chest for 6 mo, on/off

- PMH

- PE: forceful apical impulse, harsh systolic murmur at L sternal border; LVH on echo

HCM?

11

What demographic does Hypertrophic CM typically affect?

HCM often affects younger age group

12

What are complications/outcomes of HCM?

Commonly causes sudden cardiac death in young asymptomatic person (arrhythmia)

- If symptoms are present, usually have diastolic dysfunction with extertional dypsnea, angina, or non-anginal chest pain, palpitations, syncope

- Harsh systolic ejection murmur

13

Causes of HCM?

- Genetic (involves sarcomere proteins)

- 2ndary: grossly hypertrophic but microscopically minimal change (as in HTN)

14

Gross features of HCM?

- Massive myocardial hypertrophy without ventricular dilation

- On cross-section,

15

Microscopic features of HCM?

- Extensive myocyte hypertrophy

- Haphazard disarry of bundles of myocytes or individual myoctes (myocyt

- Disarray of contractile elements in sarcomeres within cells (myofiber disarray)

- Interstitial fibrosis

16

Case)

- 65 yo female with mild, intermittent chest pain

- SOB, tired no other problems

- PE: pitting edema of legs, hepatomegaly

- ECG: non-specific ST-T changes and low voltage

- Echo: reduced LV function, large atria

RCM

17

Characteristics of Restrictive CM?

- Reduced ventricular compliance an diastolic dysfunction

- Dyspnea, edema, fatigue

- Congestive hepatomegaly, elevated JVP, systolic murmurs from mitral/tricuspid regurgitation

18

What causes RCM?

- Deposition (amyloidosis, hemosiderin in hemochromatosis, etc.)

- Scar formation (radiation, endmyocardial fibrosis)

- Infiltrative (sarcoidosis, hypereosinophilic syndrome)

19

What is seen microscopically in RCM?

- Variable myocardial fibrosis

- Specific findings

20

What are some other cardiomyopathies?

- Arrhythmogenic right ventricular cardiomyopathy

- Noncompaction cardiomyopathy

21

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