HCM Flashcards
Hypertropic CM
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…
HCM Indications for ICD
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
HCM Murmur
Systolic murmur LLSB
Louder in strain valsalva
softer with squatting
HCM screening
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…
Amyloidosis
normal or low voltage (should be high voltage with thick walls if HCM)
thickened walls on ECG
ICD for HCM
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
ICD indications
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
