cardiomyopathy

Question 1

3 types of cardiomyopathy

Answer 1

1. dilated/congestive
2. hypertrophic
3. restrictive/obliterative

Question 2

progressive cardiac dilatation and contractile dysfunction usually with concomitant hypertrophy

Answer 2

dilated cardiomyopathy

Question 3

dilated cardiomyopathy characterized by (3)

Answer 3

1. global left ventricular systolic dysfunction
2. increased left ventricular cavity diameter
3. absence of HTN, valve disease, or significant coronary artery disease

Question 4

etiology of dilated cardiomypathy

Answer 4

1. AD in a cytoskeletal myocyte proteins of the sarcolemma and nuclear membrane
2. mutations in TTN
3. X-linked cardiomyopathy- dystrophin
4. viruses- CoxB or protease 2A
5. Immunologic abn.
6. toxins
7. peripartum
8. alcohol
9. iron overload
10. supraphysiologic stress

Question 5

what is the first thing that happens with dilated cardio.

Answer 5

left ventricular dilatation

Question 6

gross pathology in dilated cardio.

Answer 6

1. weight tripled
2. four chamber dilatation
3. myocardium is flabby and pale with subendocardial scars

Question 7

characterized by atrophic and hypetrophic myocardial fibers and interstitial fibrosis of the myocardium

Answer 7

dilated cardiomyopathy

Question 8

arrhythmogenic right ventricular cardiomyopathy

1. etiology
2. what is defective
3. what happens
4. what is thinned and why?

Answer 8

1. inherited disease
2. defective cell adhesion proteins in the desmosomes that link adjacent cardiac myocytes
3. right ventricular failure, rhythm disturbances, ventricular tachy or fib and sudden death
4. the right ventricular wall is thinned due to loss of myocytes with extensive fatty infiltration and fibrosis

Question 9

alcoholic cardiomyopathy

1. ____ in US and Europe
2. has direct ____ effects leading to
3. who affected
4. chronic alcoholism associated with

Answer 9

1. most common identifiable cause of DCM
2. has direct toxic effects leading to chronic progressive cardiac dysfunction, which may be fatal
3. usually men 30-50 yr. old with progressive and binge drinking
4. chronic alcoholism associated with vit. B1 deficiency or beri-beri heart

Question 10

cardiomyopathy of pregnancy

1. US vs africa
2. cause
3. risk period
4. risk factors
5. prognosis

Answer 10

1. uncommon in US vs Africa is seen up to 1%
2. cause is poorly understood but is associated with HTN, volume overload, nutritional deficiencies may be involved
3. risk period- third trimester to 6 months post partum
4. risk factors- multiparity, age>30 yr., black race and post-partum HTN
5. prognosis- 50% die due to CHF and embolisms the other 50% slowly recover. there is a high probability of recurrence with new pregnancy

Question 11

drug-induced cardiomyopathy:

1. toxicity of
2. toxicity if
3. pathology

Answer 11

1. antracycline toxicity: adriamycin and daunorubicin
2. dose dependent toxicity
3. chronic irreversible degeneration of myocytes with myofibrillar loss and vacuolar degeneration of myocytes leading to depressed contractility

Question 12

uncommon condition, showing cardiac hypertrophy out of proportion to the load of the heart

Answer 12

hypertrophic cardiomyopathy

Question 13

hypetrophic cardiomyopathy:

1. prevalence in US
2. familial
3. course
4. what happens
5. common presentation

Answer 13

1. 1/500
2. AD- point mutation of genes that encode sarcomeric proteins and a single missense point mutation of the B-myosin heavy chain on chromosome 14 which accounts for one third to one half of the cases
3. clinically variable
4. decreased left ventricular compliance. reduced chamber size resulting from impaired diastolic filling
5. young males in afflicted families with sudden cardiac death

Question 14

hypetrophic cardiomyopathy pathology (3)

Answer 14

1. heart is enlarged
2. wall of the ventricle is thick, with a small cavity and large left atrium
3. septal thickening with asymmetry thicker than left ventricular wall

Question 15

primary decrease in ventricular compliance resulting in impaired ventricular filling during diastole

Answer 15

restrictive cardiomyopathy

Question 16

restrictive cardiomyopathy

1. decreased ventricular compliance secondary to
2. may mimic

Answer 16

1. decreased ventricular compliance secondary to infiltration of the myocardium
2. may mimic constrictive pericarditis

Question 17

restrictive cardiomyopathy etiology (6)

Answer 17

1. amyloidosis
2. sarcoidosis
3. storage diseases, including hemochromatosus
4. post-radiation fibrosis
5. endomyocardial disease
6. metastatic tumors

Question 18

amylodosis restrictive cardio

1. etiology
2. what do we see

Answer 18

1. senile cardiac amyloidosis and isolated atrial amyloidosis
2. cardiomegaly is prominent with thickening of the myocardial wall and decreased motion

Question 19

generalized forms of amyloidosis

Answer 19

the heart is usually affected with right sided symptomatology- with the amyloid light chain protein is made of complete immunoglobulin light chains or their fragments ***

** is one of the three most common biochemically distinct forms of amyloid proteins that have been identified

Question 20

in senile cardiac amyloidosis the proteins deposits derive from

Answer 20

transthyretin (prealbumin) a normal serum protein produced by the liver

Question 21

abnormal iron deposition present in myocytes, in the perinuclear region of the cytoplasm

Answer 21

hemochromatosis

Question 22

secondary hemosiderosis

Answer 22

excessive blood transfusions, hemolytic anemia, increased iron intake

Question 23

non-necrotizing granulomatous inflammation in many organs specially lung and spleen

Answer 23

sarcoidosis

Question 24

sarcoidosis is restrictive or dilated?

Answer 24

both

Question 25

sudden death of sarcoidosis

Answer 25

arrhythmia