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Flashcards in Miscellaneous Heart Disease Deck (42)
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1
Q

diverse group of heart diseases involving the _____

A

myocardium

2
Q

What are the 3 functional classes of cardiomyopathy

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

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

A

hypertrophic cardiomyopathy

4
Q

epidemiology of hypertrophic cardiomyopathy

A

males in mid 20s

5
Q

Pathophys of hypertrophic cardiomyopathy

A

-dec compliance and inc LV, LA, and pulmonary pressures

30% also have obstruction due to rapid flow and venturi forces ???

6
Q

Gross pathology of hypertrophic cardiomyopathy

A

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

7
Q

Microscopic pathology of hypertrophic cardiomyopathy

A

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

interstitial and replacement fibrosis in a haphazard plexiform pattern

8
Q

Symptom of hypertrophic cardiomyopathy

A
***very similar to aortic stenosis: but these pts are much younger!!!!***
dyspnea
angina
syncope
lightheadedness
sudden death
9
Q

Signs of hypertrophic cardiomyopathy

A

S4

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

10
Q

Tx of hypertrophic cardiomyopathy

A
  • 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
Q

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

A

concentric hypertrophy vs asymmetrical hypertrophy

12
Q

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

A

cardiac amyloid

13
Q

epidemiology of cardiac amyloidosis

A

older pts

14
Q

gross pathology of cardiac amyloidosis

A

enlarged, waxy heart

15
Q

microscopic pathology of cardiac amyloidosis

A

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
Q

What type of amyloid is deposited in cardiac amyloidosis

A

P, A

**can be target for immuno stain

17
Q

symptoms of of cardiac amyloidosis

A

similar to CHF (left and right)

18
Q

signs of of cardiac amyloidosis

A

FAMILIAL

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

~left and right heart failure

19
Q

Diagnosis of of cardiac amyloidosis

A

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

ITS FAMILIAL

20
Q

multiorgan inflammatory disease with may be autoimmune

A

sarcoidosis

21
Q

epidemiology of cardiac sarcoidosis

A
young adults (20s and 30s)
AA > caucasians
22
Q

symptoms pf of cardiac sarcoidosis

A

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

AFFECTS LUNGS (dyspnea)

23
Q

pathology of cardiac sarcoidosis

A

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
Q

complications of cardiac sarcoidosis

A

arrhythmias (VT) and sudden death

25
Q

precipitation of cytoskeletal proteins

A

astroid bodies (common in sarcoidosis biopsy)

In giant cell or by themselves???

26
Q

sign of cardiac sarcoidosis

A

usually have arrhythmias

27
Q

treatment of cardiac sarcoidosis

A

steroids and immunosuppresive therapy

cardiac??

28
Q

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

A

idiopathic cardiomyopathy

29
Q

Epidemiology of idiopathic cardiomyopathy

A

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

30
Q

gross pathology of idiopathic cardiomyopathy

A

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

31
Q

microscopic pathology of idiopathic cardiomyopathy

A
  • myocyte atrophy, irregular hypertrophy
  • interstitial and perivascualr fibrosis
  • or healthy looking
32
Q

complications of idiopathic cardiomyopathy

A

mural thrombi and emboli
mitral and tricuspid regurg
arrythmias
sudden death

33
Q

diagnosis of idiopathic cardiomyopathy

A

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

34
Q

BENIGN gelatinous mesenchymal neoplasms of the endocardium

A

cardiac myxomas

35
Q

epidemiology of cardiac myxomas

A

women, rare

36
Q

Where in the heart do cardiac myxomas form?

A

left atrium

37
Q

gross path of cardiac myxomas

A

pedunculated polypoid soft gelatinous tumor, friable, focally hemorrhagic

38
Q

microscopic path of cardiac myxomas

A

? stellate cells

39
Q

symptoms of cardiac myxomas

A
non-specific...
SOB
orthopnea
cough, hemoptysis
fatigue fever
40
Q

signs of cardiac myxomas

A

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
Q

complications of cardiac myxomas

A

mitral obstruction embolization

42
Q

Tx of cardiac myxomas

A

surgical excision (curative)