Hypertrophic Cardiomyopathy Flashcards
who should get screen for HCM?
ALL first-degree relatives w/ echocardiography
when should screening begin for HCM?
12 yoa
HCM description
- uncommon
- primary cardiac disease
- diffuse or focal myocardial hypertrophy
how is HCM inherited?
autosomal DOMINANT, 50% penetration
how do HCM pts present?
- syncope (often arrhythmogenic)
- chest pain
- sudden cardiac death
syncope in HCM pts may present in which 2 ways?
- exertional syncope
- syncope a/w volume depletion
DON’T BE TRICKED
- ALL pts w/ HCM should receive
genetic counseling
distinguishing HCM from AS:
HCM
- carotid pulse
rises briskly, then declines, followed by second rise (pulsus bisferiens)
distinguishing HCM from AS:
HCM
- ejection sound
none
distinguishing HCM from AS:
HCM
- aortic regurgitation
none
distinguishing HCM from AS:
HCM
- Valsalva maneuver
increased murmur intensity
distinguishing HCM from AS:
HCM
- squatting to standing position
increased murmur intensity
distinguishing HCM from AS:
HCM
- standing to squatting position
decreased murmur intensity
distinguishing HCM from AS:
HCM
- carotid radiation
none
distinguishing HCM from AS:
HCM
- apex beat
“triple ripple”
distinguishing HCM from AS:
AS
- carotid pulse
rises slowly and has low volume (pulsus parvus et tardus)
distinguishing HCM from AS:
AS
- ejection sound
present
distinguishing HCM from AS:
AS
- aortic regurgitation
may be present
distinguishing HCM from AS:
AS
- Valsalva maneuver
decreased murmur intensity
distinguishing HCM from AS:
AS
- squatting to standing position
decreased murmur intensity
distinguishing HCM from AS:
AS
- standing to squatting position
increased murmur intensity
distinguishing HCM from AS:
AS
- carotid radiation
usually present
distinguishing HCM from AS:
AS
- apex beat
sustained single
ECG findings for HCM
- LVH
- LAE
- deeply inverted, symmetric T waves in leads V3-V6 (apical hypertrophic form)
diagnostic technique of choice for HCM
echocardiogram
pts w/ HCM should avoid
strenuous exercise
first-line tx for HCM w/ EF of 50% or more, dyspnea, and/or CP
BBs
can CCBs (verapamil) be substituted for BBs for HCM tx?
yes
third-line agent that can be added if sxs and significant outflow gradient persist
disopyramide
HCM tx used ONLY if systolic dysfunction is present
ACEIs
tx for HCM and AF
WARFARIN for ALL pts w/ HCM and AF regardless of CHADS2 score
when is surgery or septal ablation for HCM indicated?
- outflow tract gradient > 50 mmHg
- persistent sxs despite max meds
sudden death risk factors in HCM
- 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
when are HCM pts high risk for sudden death?
1 or more major risk factors
HCM pts who are high-risk for sudden death are candidates for
ICD
DON’T BE TRICKED
- are ECGs useful in predicting sudden cardiac death?
NO
DON’T BE TRICKED
- which meds should NOT be prescribed and why?
- digoxin
- vasodilators
- diuretics
- they increase LV outflow obstruction
what condition can be difficult to differentiate from HCM?
athlete’s heart (syndrome of myocardial hypertrophy)
clinical features distinguishing HCM from athlete’s heart:
HCM
- family history
positive
clinical features distinguishing HCM from athlete’s heart:
HCM
- ECG
- pathologic Q waves
- TWIs
- conduction defects
clinical features distinguishing HCM from athlete’s heart:
HCM
- doppler echocardiography
diastolic filling abnormalities
clinical features distinguishing HCM from athlete’s heart:
HCM
- extent of hypertrophy
> 15 mm
clinical features distinguishing HCM from athlete’s heart:
HCM
- pattern of hypertrophy
- asymmetric
- concentric
- eccentric
clinical features distinguishing HCM from athlete’s heart:
HCM
- LV end-diastolic dimension
< 45 mm
clinical features distinguishing HCM from athlete’s heart:
HCM
- genetic testing
positive
clinical features distinguishing HCM from athlete’s heart:
athlete’s heart
- family history
negative
clinical features distinguishing HCM from athlete’s heart:
athlete’s heart
- ECG
normal
clinical features distinguishing HCM from athlete’s heart:
athlete’s heart
- doppler echocardiography
normal diastolic filling
clinical features distinguishing HCM from athlete’s heart:
athlete’s heart
- extent of hypertrophy
12 mm or less
clinical features distinguishing HCM from athlete’s heart:
athlete’s heart
- pattern of hypertrophy
concentric
LV end-diastolic dimension
> 55 mm
clinical features distinguishing HCM from athlete’s heart:
athlete’s heart
- genetic testing
negative