Miscellaneous Disease

Question 1

What are cardiomyopathies

Answer 1

A heterogenous group of diseases associated with electrical and/or mechanical dysfunction of the heart

Question 2

What is the functional classification

Answer 2

Dilated cardiomyopathy, hypertrophic cardiomyompathy, Restrictive Cardiomyopathy

Question 3

Dilated cardiomyopathy is characterized by?

Answer 3

Impaired contractility and systolic function

Question 4

Hypertrophic and restrictive characterized by?

Answer 4

Impaired compliance and diastolic function

Question 5

Decompensation can cause what

Answer 5

Hypertrophic or restrictive cardiomyopathy to take on features of dilated cardiomyopathy

Question 6

Hypertrophic cardiomyopathy

Answer 6

group of genetic diseases characterized by hypertrophy as a compensatory mechanism for defects in myocyte contractile aparatus

Question 7

Restrictive cardiomyopathy

Answer 7

The prototype is amyloidosis. Fibrosis (due to radiation, sarcoidosis, etc) or hemochromatosis can cause it as well.

Question 8

Hypertrophic cardiomyopathy is called hypertrophic obstructive cardiomyopathy when:

Answer 8

it features asymmetrical septal hypertrophy and subaortic stenosis

Question 9

Most common cause of death in young athletes

Answer 9

hypertrophic cardiomyopathy

Question 10

Male or female predominance in hypertrophic cardiomyopathy

Answer 10

female

Question 11

Hypertrophic cardiomyopathy is 50% auto dominant and 50% from new mutations

Answer 11

truth

Question 12

The decreased comliance in hyper cardiomyo causes

Answer 12

increased left ventricular pressure, increased left atrial pressure, increased pulmonary pressure

Question 13

Microscopy shows what in hypertrophic cardiomyopathy

Answer 13

myocyte disarray, myocyte hypertrophy, interstitial and replacement fibrosis

Question 14

Complications of hypertrophic cardiomyopathy are

Answer 14

heart failure, infective endocarditis, atrial fib, left atrial mural thrombus, syncope, sudden death

Question 15

Symptoms of HC

Answer 15

dyspnea, syncope, angina,

Question 16

Signs of HC

Answer 16

S4 heart sound, crescendo decrescendo murmur at left sternal border

Question 17

hypertrophic cardiomyopathy is the most common cause of death for athletes under 35

Answer 17

Yeah, Hypertrophic atherosclerosis is the most common cause for athletes over 35

Question 18

Cardiac Amyloidosis is what kind of cardiomyopathy

Answer 18

Restrictive

Question 19

Cardiac amyloidosis due to what>

Answer 19

Deposition of abnormal protein in the interstitium of the heart

Question 20

What the hell is amyloid

Answer 20

Amyloids are fibrillar proteins produced by plasma cells or hepatocytes… Three of the most common are Ig Light chains, Transthyretin, and amyloid A

Question 21

Senile Amyloidosis

Answer 21

Transthyretin deposits, limited to the heart, present in 25% over 80

Question 22

Transthyretin amyloidosis is common in who>

Answer 22

African Americans, it is familial and autosomal dominant

Question 23

Gross pathology of transthyretin amyloidosis

Answer 23

Enlarged, waxy tan heart

Question 24

Microscopic pathology of transthyretin amyloidosis

Answer 24

Hyaline eosinophilic material. Orange on Congo Red Stain

Question 25

Complications of cardiac amyloidosis

Answer 25

Heart Failure, Heart Block, arrhythmias

Question 26

Signs of CA

Answer 26

JVD, Peripheral edema, ascites, Enlarged Liver, Pulmonary Rales

Question 27

Diagnosis of CA

Answer 27

biopsy

Question 28

Whats problematic about the treatment of amyloidosis

Answer 28

Transplantation is the only treatment, but the amyloidosis will come back unless the myeloma producing the abnormal protein is cured or the liver producing the abnormal protein is replaced.

Question 29

What the hell is sarcoidosis

Answer 29

Multi-organ inflammatory disease that may be autoimmune

Question 30

Sarcoidosis common in who?

Answer 30

African Americans. Usually presents in young adults with fever, malaise, fatigue, weight loss, dyspnea.

Question 31

Sarcoidosis favors what part of the heart

Answer 31

Base of the heart, conduction system

Question 32

Most common presentation of sarcoidosis

Answer 32

arrhythmias causing syncope or sudden death

Question 33

What the hell is Idiopathic dilated cardiomyopathy

Answer 33

A wastebasket category of nonspecific end stage heart disease with no cause evident

Question 34

Gross pathology of dilated cardiomyopathy

Answer 34

flabby heart with four chamber dilatation and commonly with mural thrombi.

Question 35

Dilated cardiomyopathy is a diagnosis of exclusion

Answer 35

Truth. SIgns and symptoms are just like heart failure

Question 36

Cardiac Myxoma

Answer 36

Benign mesenchymal neoplasm of the endocardium

Question 37

Most common primary tumor of the heart in adults (still rare) is?

Answer 37

Cardiac myxoma

Question 38

Most cardiac myxomas are located where?

Answer 38

90% in atria

Question 39

Symptoms of Cardiac myxoma

Answer 39

dyspnea, cough, transient neurological symptoms, fatigue and fever. Loud first heart sound

Question 40

Treatment of sarcoidosis

Answer 40

steroids