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Flashcards in Miscellaneous Heart Disease Deck (42):
1

diverse group of heart diseases involving the _____

myocardium

2

What are the 3 functional classes of cardiomyopathy

1. dialed (imparted contractility ad systolic function
2. hypertrophic ???
3. restrictive ??

3

a group of genetic diseases sharing hypertrophy as a compensatory mechanisms for mutations in cardiac contractile proteins

hypertrophic cardiomyopathy

4

epidemiology of hypertrophic cardiomyopathy

males in mid 20s

5

Pathophys of hypertrophic cardiomyopathy

-dec compliance and inc LV, LA, and pulmonary pressures
30% also have obstruction due to rapid flow and venturi forces ???

6

Gross pathology of hypertrophic cardiomyopathy

thickened wall
asymmetric hypertrophy of upper septum and anterior LV
thickened atrial leaflet of mital valve???

7

Microscopic pathology of hypertrophic cardiomyopathy

myocyte disarray (oriented in diff directions**cannot contract as a unit anymore) and hypertrophy

interstitial and replacement fibrosis in a haphazard plexiform pattern

8

Symptom of hypertrophic cardiomyopathy

***very similar to aortic stenosis: but these pts are much younger!!!!***
dyspnea
angina
syncope
lightheadedness
sudden death

9

Signs of hypertrophic cardiomyopathy

S4
rough crescendo-decrescendo systolic murmur at left sternal boarder that inc with standing or valvsa maneuver

10

Tx of hypertrophic cardiomyopathy

-BB
-CCB
-amiodarone
-surgical resection of hypertrophied septum
-injection of poison into first septal perforator to infarct hypertrophied septum
-implanted defibrillator

STOP PLAYING COMPETITIVE SPORTS (can jog)

11

How is the hypertrophy as compensation for HTN or valvualr disease different than the hypertrophy due to genetic disease?

concentric hypertrophy vs asymmetrical hypertrophy

12

due to deposition of abnormal protein in the interstitium of the heart

cardiac amyloid

13

epidemiology of cardiac amyloidosis

older pts

14

gross pathology of cardiac amyloidosis

enlarged, waxy heart

15

microscopic pathology of cardiac amyloidosis

intertilial depostion of hyalin smooth eosinaphillic material
** initially in blood vessels

congo red + apple green bifringence

diff than collagen bc it is smooth (not wavy)

16

What type of amyloid is deposited in cardiac amyloidosis

P, A

**can be target for immuno stain

17

symptoms of of cardiac amyloidosis

similar to CHF (left and right)

18

signs of of cardiac amyloidosis

FAMILIAL

JVD
kussmauls sign (JVD worse with ??
peripheral edema
ascities
enlarged tender liver
pulmonary rales

~left and right heart failure

19

Diagnosis of of cardiac amyloidosis

CT
MRI
commonly in multiple other organs (which you can biopsy if they are in failure as well)

ITS FAMILIAL

20

multiorgan inflammatory disease with may be autoimmune

sarcoidosis

21

epidemiology of cardiac sarcoidosis

young adults (20s and 30s)
AA > caucasians

22

symptoms pf of cardiac sarcoidosis

non-specific: fatigue, malaise, weight loss etc
+ syncope

AFFECTS LUNGS (dyspnea)

23

pathology of cardiac sarcoidosis

granulomas in LUNGS, lymph nodes, liver spleen, BM, skin, eyes and HEART
-giant cell and eventual fibrosis
-astroid body

favors the base of the heart and commonly involves conduction system!!

24

complications of cardiac sarcoidosis

arrhythmias (VT) and sudden death

25

precipitation of cytoskeletal proteins

astroid bodies (common in sarcoidosis biopsy)

In giant cell or by themselves???

26

sign of cardiac sarcoidosis

usually have arrhythmias

27

treatment of cardiac sarcoidosis

steroids and immunosuppresive therapy

cardiac??

28

non-specific end stage heart disease with dilation and no cause evident

idiopathic cardiomyopathy

29

Epidemiology of idiopathic cardiomyopathy

middle age with slowly progressive heart failure but with some acute compensation

30

gross pathology of idiopathic cardiomyopathy

enlarged, soft, flabby heart
all 4 chambers symmetrically dilated
commonly have mural thrombi

31

microscopic pathology of idiopathic cardiomyopathy

-myocyte atrophy, irregular hypertrophy
-interstitial and perivascualr fibrosis
-or healthy looking

32

complications of idiopathic cardiomyopathy

mural thrombi and emboli
mitral and tricuspid regurg
arrythmias
sudden death

33

diagnosis of idiopathic cardiomyopathy

determine that the heart is dilated and then must exclude all other causes of dilation
= diagnosis of exclusion!

34

BENIGN gelatinous mesenchymal neoplasms of the endocardium

cardiac myxomas

35

epidemiology of cardiac myxomas

women, rare

36

Where in the heart do cardiac myxomas form?

left atrium

37

gross path of cardiac myxomas

pedunculated polypoid soft gelatinous tumor, friable, focally hemorrhagic

38

microscopic path of cardiac myxomas

? stellate cells

39

symptoms of cardiac myxomas

non-specific...
SOB
orthopnea
cough, hemoptysis
fatigue fever

40

signs of cardiac myxomas

simialr to mitral stenosis!!

loud first heart sound, diastolic murmur, diastolic tumor plop

mitral regurg w/ holosystolic murmur if the tumor hangs into the LV

41

complications of cardiac myxomas

mitral obstruction embolization

42

Tx of cardiac myxomas

surgical excision (curative)