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Hf Boards > Hocm > Flashcards

Hocm Flashcards

1
Q

Define hocm. Prevalence? Death

A

Lvh without cause wall thickness >15mm

1/500
1% year sudden dearh

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2
Q

Inheritance

What genes?

A

50 ad
30% bmhc 15% trop
50% do not know

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3
Q

What is a significant gradient and what dies it mean

A

> 30mmhg

Does not predict sudden death or correlate with prognosis

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4
Q

3 nonsarcomeric genes associated with cardiac hypertrophy

A

Prkag2, LAMP, GLA (fabrys)

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5
Q

Likelihood of finding a mutation in hocm

A

50%

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6
Q

How should you screen family members with hocm

A 
H&p
ECG
Echo annually in adolescence or with consideration of competitive sports. 
After 18 perform every 5 years
If athletic should perform every year.
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7
Q

Death in hocm

A

3% year
Most common cause of death in athletes
Think syncope, fh, arrhythmia, alpha troop myosin mutants

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8
Q

Rf for sudden death

A 
Syncope
Fh
Sudden death
Arrhythmias on holter
Low Bp on treadmill
Lv wall thickness >29mm
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9
Q

Sports to avoid in hocm

A

Basketball singles tennis, weights extreme sports

Avoid systemic training competitive sports

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10
Q

Most common mutant in hocm

A

Myh7 beta myosin

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11
Q

Dallas criteria
Initial?
Subsequent?

A

Initial
1 necrosis/degeneration or both
2. Infiltrate but no damage
3. Nothing

Subsequent

  1. Ongoing myocarditis
  2. Healing myocarditis
  3. Healed myocarditis
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12
Q

Outcomes in myocarditis

A

25% better
50% chronic
25% worse

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13
Q

Dx sarcoid

A

Bx proven
Extra cardiac AND 2 major
2 major and 1 minor

Major av block, thin septum, gallium , Ef<50
Minor abn EKG (vt:PVC ebb, q waves
Echo:wall motion aneurysm increased wt
Perfusion defect/Abn MRI, severe fibrosis or monocytes infiltrate

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14
Q

Define ppm

A

Hf 1 month before or 5 months after

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15
Q

7 rf for ppm

A

Age, multiparty,AA, cocaine, Htn, multiple fetus, beta agonists

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16
Q

Define familial cm

A

2 or more family members or unexplained scd <35

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17
Q

What are the muscular dystrophies

A 
Duchenne md (no dystrophin)
Beckett (decreased dystrophin
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18
Q

What chemo causes cm

A

Doxorubicin daunorubiin bleomyocin cyclophosphamide sunitinib trastuzumab

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19
Q

What antiretrovirals cause cm

A

Zidovudine didanosine zalcitabine

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20
Q

Incidence of HF with doxy

A

1.6% early5% late related to dose
7-28% with cyclophosphamide
3-11% belomycin
2% with herceptin(trazutinab) , 7% with packitaxol, 28% with doxorubicin

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21
Q

Percent of outflow tract obstruction/Sam in hocm

A

70% causes

Chest pain, dyspnea and fatigue

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22
Q

What is jet look like in Sam

A

Posterior jet

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23
Q

First line treatment of hocm

A

Stop vasodilators, including vasodilationg

Avoid Viagra etc..

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24
Q

Effect of myomectomy

A

Lowers mortality and scd rate

Ablation worse in patients above 65.

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25
Q

Drug therapy in hocm

A

Add disopyramide if on bb/ccb

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26
Q

Who should get defib

A

History of cardiac arrest or sustained vt icd reccomended
Fh of sudden death, lvh 30mm or recurrent syncope level 2a
Nsvt or Abn Bp response have other risk factors than 2a otherwise 2b
Otherwise

27
Q

Yield of genetic testing in hocm

A

Around 50%

Good for hocm, not so good for dilated cm

28
Q

How to screen families when index mutation not known

A

ECG + echo

If nothing repeat at regular intervals

29
Q

Why do genetic testing

A

Family screening

Reccomended if dcm and conduction disease or mutation is known

30
Q

Hcm family screening

A

Initial at age 10, repeat every 1-2 years until 20 then every 5 years stop at age 50-60

31
Q

Three most common mutations in hcm

A

Mybpc3, myh7, tnnt2

32
Q

Family screening for dcm

A

Age 10 q1-3 year
Age 10-20 q1-2
Age > 20 q2-5
Screening can be stopped at 50-60

33
Q

Lvnc gene? Screening

A

Myh7. Screen every 1-3 years then every 5 years after age 20

34
Q

Gene associated for restrictive cm

Arvd

A

Tnni 3.

Pkp2 Desmoplakin dsp dsg2 dsc2

35
Q

Toxin myocarditis

A

Anthracycline, cocaine, interleukin,

Sulfonamides cephalosporins

36
Q

Pathogenesis of myocarditis

A

Nk cells and macrophages

secrete il1, il2 tnf, interferon,

37
Q

4 types of amyloid

A

Al, attr, appoA1, senile (normal transthyreitin).

38
Q

Hemochromatosis

A

Primary or secondary (frequent transfusions)
Looks more dilated
Rx with chelation

39
Q

Fabrys

A

Lack of galactosidase a. X linked

40
Q

Define peripartum

A

1 month prior to 5 months after pregnancy

41
Q

Diagnosing stress cm

A

Absence of Cath Abn
Wma beyond a single territory
Recovery of fxn
Diffuse twi

42
Q

Who cant get pregnant after ppcm

A

If your ef dont recover don’t do it.

43
Q

Difference between duchanne and Becker

A

No dystrophin in duchanne

44
Q

Incidence of HF post chemo

A

Age, Htn, cad, low ef

45
Q

Doxorubicin unique?
Cyclophosphamide unique
Traztumibid

A

Early and late effects of doxorubicin
Traztuzimab only in combine
Cyclophosphamide is dose responsive

46
Q

How much doxirubicin is bad

A

550mg/m2

47
Q

Arvd epidemiology

Screening

A

.2/1000 in us , higher in Italy
Repolarization abnormalities v1-v3
Mutations pkp dsg dsp

48
Q

Physical activity in inherited disease

A

Nothing rigorous, non competitive

Can bowl, golf

49
Q

How do adriamycins work

A

Interpolate between base pairs of DNA: inhibit synthesis

Inhibits topoisomerase 2

50
Q

How to minimize cardiotoxicity of chemo

A
  1. Use low doses
  2. Use lysosomal formulations
  3. Longer infusions
  4. Use dexrazocxane
51
Q

Percentage of patients with idcm with first degree family members with disease

A

20%

52
Q

Define familial dcm

A

2 family members in a family or scd in a family member under 35

53
Q

Name 3 x linked cms

A

Beckers
Duchannes
Danon

54
Q

Define non compaction

A

Ratio of 2:1 non compacted to compacted myocardium

55
Q

Screening in dcm

A

Every 1-2 until 20 then 2-5 stop at 50.

56
Q

Guidelines for genetic testing in fdcm

Hocm

A

Dcm + conduction disease
Or known mutation
Hocm in any patients

57
Q

Common sarcomeric mutations in dcm

A

Myh7

Tnn2

58
Q

Common sarcomeric mutations in hocm

A

Mybpc3

Myh7

59
Q

Differences in genetic testing for hocm vs dcm

A

Hocm 40-70% efficacy
Dcm <20%
Arvd 30-60%

60
Q

What is threshold gradients in hocm for septal reduction.
How to elicit gradients?
What is a positive exercise test in hocm

A

> 50 peak
Exercise echo or valsalva echo. Dont need routine Cath.
SBp changes < 20.

61
Q

Five 1a guidelines for hocm icd

A
  1. secondary prevention
  2. syncope
  3. fh
  4. Septal thickness > 30
  5. Nsvt 3 beats at 120bpm
  6. Bo either fails to go up by 20 or decreases by 20
62
Q

Criteria for Arvd

A 
60% fat
Rv dysfunction
Inverted t waves V1-3
Epsilon wave v1-v3
Arrhythmia 
Fh
63
Q

2a indications for bx

A

HF > 3 months dilated, fails to respond to care

? Eosinophilia, Anthracyclines, restrictive, tumors, children

Hf Boards (11 decks)

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