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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
Drug therapy in hocm
Add disopyramide if on bb/ccb
26
Who should get defib
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
Yield of genetic testing in hocm
Around 50% | Good for hocm, not so good for dilated cm
28
How to screen families when index mutation not known
ECG + echo | If nothing repeat at regular intervals
29
Why do genetic testing
Family screening | Reccomended if dcm and conduction disease or mutation is known
30
Hcm family screening
Initial at age 10, repeat every 1-2 years until 20 then every 5 years stop at age 50-60
31
Three most common mutations in hcm
Mybpc3, myh7, tnnt2
32
Family screening for dcm
Age 10 q1-3 year Age 10-20 q1-2 Age > 20 q2-5 Screening can be stopped at 50-60
33
Lvnc gene? Screening
Myh7. Screen every 1-3 years then every 5 years after age 20
34
Gene associated for restrictive cm | Arvd
Tnni 3. | Pkp2 Desmoplakin dsp dsg2 dsc2
35
Toxin myocarditis
Anthracycline, cocaine, interleukin, | Sulfonamides cephalosporins
36
Pathogenesis of myocarditis
Nk cells and macrophages | secrete il1, il2 tnf, interferon,
37
4 types of amyloid
Al, attr, appoA1, senile (normal transthyreitin).
38
Hemochromatosis
Primary or secondary (frequent transfusions) Looks more dilated Rx with chelation
39
Fabrys
Lack of galactosidase a. X linked
40
Define peripartum
1 month prior to 5 months after pregnancy
41
Diagnosing stress cm
Absence of Cath Abn Wma beyond a single territory Recovery of fxn Diffuse twi
42
Who cant get pregnant after ppcm
If your ef dont recover don't do it.
43
Difference between duchanne and Becker
No dystrophin in duchanne
44
Incidence of HF post chemo
Age, Htn, cad, low ef
45
Doxorubicin unique? Cyclophosphamide unique Traztumibid
Early and late effects of doxorubicin Traztuzimab only in combine Cyclophosphamide is dose responsive
46
How much doxirubicin is bad
550mg/m2
47
Arvd epidemiology | Screening
.2/1000 in us , higher in Italy Repolarization abnormalities v1-v3 Mutations pkp dsg dsp
48
Physical activity in inherited disease
Nothing rigorous, non competitive | Can bowl, golf
49
How do adriamycins work
Interpolate between base pairs of DNA: inhibit synthesis | Inhibits topoisomerase 2
50
How to minimize cardiotoxicity of chemo
1. Use low doses 2. Use lysosomal formulations 3. Longer infusions 4. Use dexrazocxane
51
Percentage of patients with idcm with first degree family members with disease
20%
52
Define familial dcm
2 family members in a family or scd in a family member under 35
53
Name 3 x linked cms
Beckers Duchannes Danon
54
Define non compaction
Ratio of 2:1 non compacted to compacted myocardium
55
Screening in dcm
Every 1-2 until 20 then 2-5 stop at 50.
56
Guidelines for genetic testing in fdcm | Hocm
Dcm + conduction disease Or known mutation Hocm in any patients
57
Common sarcomeric mutations in dcm
Myh7 | Tnn2
58
Common sarcomeric mutations in hocm
Mybpc3 | Myh7
59
Differences in genetic testing for hocm vs dcm
Hocm 40-70% efficacy Dcm <20% Arvd 30-60%
60
What is threshold gradients in hocm for septal reduction. How to elicit gradients? What is a positive exercise test in hocm
>50 peak Exercise echo or valsalva echo. Dont need routine Cath. SBp changes < 20.
61
Five 1a guidelines for hocm icd
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
Criteria for Arvd
``` 60% fat Rv dysfunction Inverted t waves V1-3 Epsilon wave v1-v3 Arrhythmia Fh ```
63
2a indications for bx
HF > 3 months dilated, fails to respond to care | ? Eosinophilia, Anthracyclines, restrictive, tumors, children

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