Hypertrophic Cardiomyopathy Flashcards

1
Q

who should get screen for HCM?

A

ALL first-degree relatives w/ echocardiography

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2
Q

when should screening begin for HCM?

A

12 yoa

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3
Q

HCM description

A
  • uncommon
  • primary cardiac disease
  • diffuse or focal myocardial hypertrophy
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4
Q

how is HCM inherited?

A

autosomal DOMINANT, 50% penetration

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5
Q

how do HCM pts present?

A
  • syncope (often arrhythmogenic)
  • chest pain
  • sudden cardiac death
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6
Q

syncope in HCM pts may present in which 2 ways?

A
  • exertional syncope

- syncope a/w volume depletion

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7
Q

DON’T BE TRICKED

  • ALL pts w/ HCM should receive
A

genetic counseling

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8
Q

distinguishing HCM from AS:

HCM

  • carotid pulse
A

rises briskly, then declines, followed by second rise (pulsus bisferiens)

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9
Q

distinguishing HCM from AS:

HCM

  • ejection sound
A

none

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10
Q

distinguishing HCM from AS:

HCM

  • aortic regurgitation
A

none

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11
Q

distinguishing HCM from AS:

HCM

  • Valsalva maneuver
A

increased murmur intensity

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12
Q

distinguishing HCM from AS:

HCM

  • squatting to standing position
A

increased murmur intensity

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13
Q

distinguishing HCM from AS:

HCM

  • standing to squatting position
A

decreased murmur intensity

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14
Q

distinguishing HCM from AS:

HCM

  • carotid radiation
A

none

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15
Q

distinguishing HCM from AS:

HCM

  • apex beat
A

“triple ripple”

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16
Q

distinguishing HCM from AS:

AS

  • carotid pulse
A

rises slowly and has low volume (pulsus parvus et tardus)

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17
Q

distinguishing HCM from AS:

AS

  • ejection sound
A

present

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18
Q

distinguishing HCM from AS:

AS

  • aortic regurgitation
A

may be present

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19
Q

distinguishing HCM from AS:

AS

  • Valsalva maneuver
A

decreased murmur intensity

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20
Q

distinguishing HCM from AS:

AS

  • squatting to standing position
A

decreased murmur intensity

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21
Q

distinguishing HCM from AS:

AS

  • standing to squatting position
A

increased murmur intensity

22
Q

distinguishing HCM from AS:

AS

  • carotid radiation
A

usually present

23
Q

distinguishing HCM from AS:

AS

  • apex beat
A

sustained single

24
Q

ECG findings for HCM

A
  • LVH
  • LAE
  • deeply inverted, symmetric T waves in leads V3-V6 (apical hypertrophic form)
25
Q

diagnostic technique of choice for HCM

A

echocardiogram

26
Q

pts w/ HCM should avoid

A

strenuous exercise

27
Q

first-line tx for HCM w/ EF of 50% or more, dyspnea, and/or CP

A

BBs

28
Q

can CCBs (verapamil) be substituted for BBs for HCM tx?

A

yes

29
Q

third-line agent that can be added if sxs and significant outflow gradient persist

A

disopyramide

30
Q

HCM tx used ONLY if systolic dysfunction is present

A

ACEIs

31
Q

tx for HCM and AF

A

WARFARIN for ALL pts w/ HCM and AF regardless of CHADS2 score

32
Q

when is surgery or septal ablation for HCM indicated?

A
  • outflow tract gradient > 50 mmHg

- persistent sxs despite max meds

33
Q

sudden death risk factors in HCM

A
  • previous cardiac arrest
  • spontaneous sustained VT
  • family h/o sudden death (first-degree relative)
  • unexplained syncope
  • LV wall thickness 30 mm or more
  • blunted increase or decrease in SBP w/ exercise
  • spontaneous NSVT
  • HF that progresses to DCM and LVEF 30% or less
34
Q

when are HCM pts high risk for sudden death?

A

1 or more major risk factors

35
Q

HCM pts who are high-risk for sudden death are candidates for

A

ICD

36
Q

DON’T BE TRICKED

  • are ECGs useful in predicting sudden cardiac death?
A

NO

37
Q

DON’T BE TRICKED

  • which meds should NOT be prescribed and why?
A
  • digoxin
  • vasodilators
  • diuretics
  • they increase LV outflow obstruction
38
Q

what condition can be difficult to differentiate from HCM?

A

athlete’s heart (syndrome of myocardial hypertrophy)

39
Q

clinical features distinguishing HCM from athlete’s heart:

HCM

  • family history
A

positive

40
Q

clinical features distinguishing HCM from athlete’s heart:

HCM

  • ECG
A
  • pathologic Q waves
  • TWIs
  • conduction defects
41
Q

clinical features distinguishing HCM from athlete’s heart:

HCM

  • doppler echocardiography
A

diastolic filling abnormalities

42
Q

clinical features distinguishing HCM from athlete’s heart:

HCM

  • extent of hypertrophy
A

> 15 mm

43
Q

clinical features distinguishing HCM from athlete’s heart:

HCM

  • pattern of hypertrophy
A
  • asymmetric
  • concentric
  • eccentric
44
Q

clinical features distinguishing HCM from athlete’s heart:

HCM

  • LV end-diastolic dimension
A

< 45 mm

45
Q

clinical features distinguishing HCM from athlete’s heart:

HCM

  • genetic testing
A

positive

46
Q

clinical features distinguishing HCM from athlete’s heart:

athlete’s heart

  • family history
A

negative

47
Q

clinical features distinguishing HCM from athlete’s heart:

athlete’s heart

  • ECG
A

normal

48
Q

clinical features distinguishing HCM from athlete’s heart:

athlete’s heart

  • doppler echocardiography
A

normal diastolic filling

49
Q

clinical features distinguishing HCM from athlete’s heart:

athlete’s heart

  • extent of hypertrophy
A

12 mm or less

50
Q

clinical features distinguishing HCM from athlete’s heart:

athlete’s heart

  • pattern of hypertrophy
A

concentric

51
Q

LV end-diastolic dimension

A

> 55 mm

52
Q

clinical features distinguishing HCM from athlete’s heart:

athlete’s heart

  • genetic testing
A

negative