Miscellaneous Heart Disease

Question 1

diverse group of heart diseases involving the _____

Answer 1

myocardium

Question 2

What are the 3 functional classes of cardiomyopathy

Answer 2

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

Question 3

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

Answer 3

hypertrophic cardiomyopathy

Question 4

epidemiology of hypertrophic cardiomyopathy

Answer 4

males in mid 20s

Question 5

Pathophys of hypertrophic cardiomyopathy

Answer 5

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

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

Question 6

Gross pathology of hypertrophic cardiomyopathy

Answer 6

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

Question 7

Microscopic pathology of hypertrophic cardiomyopathy

Answer 7

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

interstitial and replacement fibrosis in a haphazard plexiform pattern

Question 8

Symptom of hypertrophic cardiomyopathy

Answer 8

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

Question 9

Signs of hypertrophic cardiomyopathy

Answer 9

S4

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

Question 10

Tx of hypertrophic cardiomyopathy

Answer 10

• 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)

Question 11

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

Answer 11

concentric hypertrophy vs asymmetrical hypertrophy

Question 12

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

Answer 12

cardiac amyloid

Question 13

epidemiology of cardiac amyloidosis

Answer 13

older pts

Question 14

gross pathology of cardiac amyloidosis

Answer 14

enlarged, waxy heart

Question 15

microscopic pathology of cardiac amyloidosis

Answer 15

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)

Question 16

What type of amyloid is deposited in cardiac amyloidosis

Answer 16

P, A

**can be target for immuno stain

Question 17

symptoms of of cardiac amyloidosis

Answer 17

similar to CHF (left and right)

Question 18

signs of of cardiac amyloidosis

Answer 18

FAMILIAL

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

~left and right heart failure

Question 19

Diagnosis of of cardiac amyloidosis

Answer 19

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

ITS FAMILIAL

Question 20

multiorgan inflammatory disease with may be autoimmune

Answer 20

sarcoidosis

Question 21

epidemiology of cardiac sarcoidosis

Answer 21

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

Question 22

symptoms pf of cardiac sarcoidosis

Answer 22

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

AFFECTS LUNGS (dyspnea)

Question 23

pathology of cardiac sarcoidosis

Answer 23

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!!

Question 24

complications of cardiac sarcoidosis

Answer 24

arrhythmias (VT) and sudden death

Question 25

precipitation of cytoskeletal proteins

Answer 25

astroid bodies (common in sarcoidosis biopsy) In giant cell or by themselves???

Question 26

sign of cardiac sarcoidosis

Answer 26

usually have arrhythmias

Question 27

treatment of cardiac sarcoidosis

Answer 27

steroids and immunosuppresive therapy cardiac??

Question 28

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

Answer 28

idiopathic cardiomyopathy

Question 29

Epidemiology of idiopathic cardiomyopathy

Answer 29

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

Question 30

gross pathology of idiopathic cardiomyopathy

Answer 30

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

Question 31

microscopic pathology of idiopathic cardiomyopathy

Answer 31

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

Question 32

complications of idiopathic cardiomyopathy

Answer 32

mural thrombi and emboli mitral and tricuspid regurg arrythmias sudden death

Question 33

diagnosis of idiopathic cardiomyopathy

Answer 33

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

Question 34

BENIGN gelatinous mesenchymal neoplasms of the endocardium

Answer 34

cardiac myxomas

Question 35

epidemiology of cardiac myxomas

Answer 35

women, rare

Question 36

Where in the heart do cardiac myxomas form?

Answer 36

left atrium

Question 37

gross path of cardiac myxomas

Answer 37

pedunculated polypoid soft gelatinous tumor, friable, focally hemorrhagic

Question 38

microscopic path of cardiac myxomas

Answer 38

? stellate cells

Question 39

symptoms of cardiac myxomas

Answer 39

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

Question 40

signs of cardiac myxomas

Answer 40

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

Question 41

complications of cardiac myxomas

Answer 41

mitral obstruction embolization

Question 42

Tx of cardiac myxomas

Answer 42

surgical excision (curative)