Hypertrophic Cardiomyopathy Flashcards
Symptoms of hypertrophic cardiomyopathy
HF
Syncope (LVOT obstruction)
AF
Angina (thick myocardium therefore high O2 demands but intermyocardial compression of coronary arteries)
Sudden death
Genetics of HCM
Autosomal dominant
2 main genes
1. beta myosin heavy chain
2. Myosin binding protein C
variable penetrance
Why may sudden death occur
LVOT obstruction (catecholamine driven)
MI
VF arrest as AF transmitted via accessory pathway
Clinical signs of HCM (non auscultation)
Bifid pulse
jerky pulse
AF
Giant a waves if pulmonary HTN
Laterally displaced heaving apex
double impulse
Auscultation of HCM
ESM
- louder when standing or Valsalva
- quieter on squatting
loud S1
S4
Associated conditions
WPW
Friedrichs ataxia
Phaemochromocytoma
Mnemonic of HCM
MR SAM ASH
Mitral
Regurg
Systolic
Anterior
Motion of MV leaflet
ASymmetrical hypertrophy
ECG in HCM
LVH
P-Mitrale
Marked lateral TWI (deep in precordial leads)
Deep lateral Q waves
VT
Investigations of HCM
ECG
CXR
TTE/ exercise echo
Cardiac MRI
Genetic testing
CXR in HCM
May be normal/ cardiomegaly/ LA/LV enlargement
Poor prognostic signs of HCM
Young
Syncope
Hypotension on exercise
FHx of sudden death
Massive LVH
Apical aneurysm
What things should be avoided in HCM
Anything that may make the heart smaller (i.e. reduce afterload/preload/ worsening LVOT obstruction) or increase inotropy
Medications: ACEI/ Nitrates/ careful diuretic use/ digoxin
Lifestyle: strenuous exercise (esp weight lifting, dehydration)
How can HCM be treated medically?
ABCDE
Amiodarone
Betablockers
Cardiac catheritisation/ CCBs (cardiac selective)
Duel chamber PPM/ Disopyramide
Endocarditis prophylaxis
Interventional treatment of HCM?
Alcohol septal ablations
surgical myomectomy of LVOT
ICD insertion
