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Flashcards in Cardiomyopathy Deck (35):
1

Definition of Cardiomyopathy

Structural or functional abnormality of the myocardium, independent of any other valvular, coronary, or myocardial involvement in systemic disease.

2

Dilated Cardiomyopathy

Dilated LV, cardiomegaly, impaired systolic function, increased myocardial mass, increased predisposition of intra-cardiac thrombi.

3

Etiology of DCM

Idiopathic, viral (Coxsackie B, Parvovirus B19), Sarcoidosis, chronic alcohol abuse, hypothyroidism, muscular dystrophy.

4

Effect of DCM on EDP?

Decreases EDP and decreases CO -- LV can't squeeze blood out. Decrease in renal perfusion, increases in RAAS, which increases PVR and causes dilation of all 4 chambers and causes CHF.

5

Symptoms of DCM?

CHF! Dyspnea, orthopnea, PND, peripheral edema, fatigue.

6

Signs of DCM

Gallop rhythm, rales, JVD, edema, tachycardia

7

Xray of DCM shows

Cardiomegaly and signs of HF (boggy lungs)

8

EKG of DCM shows

Nothing specific, can show a number of abnormalities.

9

Echo of DCM shows

Chamber dilatation. Very valuable.

10

Cardiac cath of DCM

Do a biopsy to confirm diagnosis.

11

Treatment of DCM

Diuretics to reduce preload
ACEI/ARB
Beta Blocker (actually upregulates B receptors)
Aldosterone antagonists
Last 3 actually reduce mortality

Also need to anticoagulate

12

Hypertrophic cardiomyopathy

Assymetric hypertrophy of the LV septum (not caused by chronic pressure overload)

13

Etiology of hypertrophic cardiomyopathy?

Autosomal dominant inheritance, but much fewer people develop HCM than have the mutation, so clearly some epigenetic factors.

14

Pathology of HCM and disease states

Myofiber disarray -> Ventricular arrythmias --> Sudden death or syncope

LVH --> Impaired relaxation --> Increased filling pressure --> Dyspnea, also increased myocardial oxygen demand, and angina

Dynamic LV outflow obstruction --> increased systolic pressure, mitral regurg, failure to increase CO with exertion. Causes dyspnea

15

Symptoms of HCM

Dyspnea, angina, syncope, sudden cardiac death

16

Signs of HCM

S4 (ventricle is stiff, atria need to contract hard to fill)

17

Effect of Valsalva on HCM systolic murmur

Gets louder because venous return reduces, and murmur of obstruction increases

18

Effect of squatting on HCM systolic murmur

gets quieter because increased venous return

19

Describe HCM murmur

Crescendo-decrescendo at LLSB

20

Best diagnostic test for HCM

Echo, will show asymmetrical hypertrophy of the LV septum

21

Treatment of HCM?

Beta Blockers- will decrease myocardial oxygen demand, decrease LV outflow gradient, increase diastolic filling, decrease frequency of bad ventricular beats.

Calcium channel blockers
Diuretics for overt CHF
Amiodarone for arrythmias

22

What to avoid with HCM

Vasodilators and digoxin.

23

Restrictive cardiomyopathy

Less common than the other two. Diastolic dysfunction with preserved systolic function. Causes horrible CHF.

24

Causes of RCM

Noninfiltrative -- idiopathic

Infiltrative -- amyloidosis, sarcoidosis,

Storage- hemochromatosis

Tropical environment

25

Most common etiology of RCm

Amyloidosis (AL) -- deposition of the Ig light chain fragments secreted by a plasma cell tumor (multiple myeloma). --> primary.

Secondary amyloidosis is from Rheumatoid arthritis.

Deposition is extracellular.

26

Pathophysiology of RCm

Increased diastolic ventricular pressure causes venous congestion.

Decreased ventricular filling causes decreased CO, causing weakness and fatigue.

27

Kussmaul sign

Increased JVD with inspiration. Shows diastolic dysfunction

RCM has symptoms of both right and left heart failure.

28

Pathology of viral dilated cardiomyopathy?

White spots on the outside of heart, congested vasculature.

Histology reveals lymphocytes.

29

Aspergillus infection of the heart

Acute angle braching of septate hyphae

30

Chagas disease pathology of the heart?

Amastigotes within the cytoplasm. Trypanosoma cruzi transmitted by the Reduviid bug.

31

Giant cell myocarditis

Causes granuloma formation with multinucleated giant cells.

32

Sarcoidosis of heart

Patchy white discoloration of LV, noncaseating granulomas with multinucleated giant cells.

33

Pathologic feature of hypersensitivity related dilated cardiomyopathy?

Eosinophils

34

How does alcohol cause dilated cardiomyopathy?

Direct toxic effect of alcohol and formaldehyde

Thiamine deficiency

Cobalt additive

No specific histologic features.

35

Microscopic features of Hypertrophic Cardiomyopathy?

Myofibers are completely disarrayed with interstitial fibrosis.

Crissed-crossed myofibrils.