HCM Flashcards

1
Q

Hypertropic CM

A

autosomal dominant
sarcomere proteins
If pathologic gene identified - > SCREEN FAMILY
If no gene identified no need for screening family (genetically)

Holter HCM patients if symptoms (ie palpitations, syncope)

HCM patient with 1st deg relative SCD -> echo non-dx - need cardiac MR to rule out atypical areas of LVH…

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2
Q

HCM Indications for ICD

A

Secondary prevention

1) Personal hx of SCD
2) VF
3) Hemodynamically significant VT (hypotensive response to excercise)

Primary prevention

4) First degree relative with SCD
5) Max wall thickness >30mm
6) One or more syncopal episodes (hypotensive response to excercise)

If NSVT or abn BP response to excercise also need

1) LVOT obstruction gradient >30mm Hg (severe LVH)
2) Gadolinium enhancement on MR
3) LV apical aneurysm

**NO ROLE FOR EPS in decision of primary prevention of ICD
NO ICD for..
-fhx syncope
-NSVT alone without other risk factors

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3
Q

HCM Murmur

A

Systolic murmur LLSB
Louder in strain valsalva
softer with squatting

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4
Q

HCM screening

A

Echo then Cardiac MR (if hypertrophy in place other than septum as heterogenious phenotypic expression)

Genetic testing of index relative first - then genotype that mutation in patient if positive…

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5
Q

Amyloidosis

A

normal or low voltage (should be high voltage with thick walls if HCM)
thickened walls on ECG

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6
Q

ICD for HCM

A

Primary prevention

1) prior SCD
2) VF
3) hemodynamically significant VT

or secondary prevention

1) SCD in 1st deg relatively 2/2 HCM
2) LV wall >30mm
3) one or more recent unexplained syncopal episodes
4) NSVT with (needs holter)
a) LVOT gradient >30mmHg
b) LV apical aneuysm
c) double/compound genetic mutations
d) MRI with late gad enhancment

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7
Q

ICD indications

A

1) FHx premature HCM related death
2) Unexplained syncope
3) NSVT on holter
4) hypotensive BP response stress test
5) Extreme LVH >30mm

If none of these - then ok to do stress test to see if hypotensive response to excercise

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