Hypertrophic Cardiomyopathy Flashcards
What is hypertrophic cardiomyopathy (HCM)?
LV outflow tract obstruction from asymmetric septal hypertrophy.
It is the leading cause of sudden cardiac death in the young.
The prevalence is 0.2% (1:500).
It can be either sporadic (50%) or inherited autosomal dominantly.
The inheritance involves b-myosin, a-tropomyosin and troponin T.
It is important to ask about sudden death at a young age in FH.
Clinical features of HCM.
Sudden death might be the first presentation.
Extertional syncope or pre-syncope
Angina
Dyspnoea
Palpitations
Syncope
CCF
Jerky pulse
Double-apex beat
Harsh ejection systolic murmur
Investigations of HCM.
ECG
Echo
MRI
Cardiac catheterisation
Exercise test +/- holter monitoring
ECG findings in HCM.
LVH
Progressive T-wave inversion
Deep Q waves in inferior and lateral leads.
AF
WPW syndrome
Ventricular ectopics
VT
Echo findings in HCM.
Asymmetrical septal hypertrophy
Small LV cavity
Why might a cardiac catherisation be done?
To assess severity of disease.
However it can cause VT
Management of HCM.
B-blockers or verapamil for symptoms.
Amiodarone for arrhythmias
Anticoagulation for paroxysmal AF or systemic emboli
Septal myomectomy is reserved for those with severe symptoms.
Consider ICD
Prognosis of HCM.
- 9%/yr if < 14 yo at presentation
- 5%/yr if > 14 yo
Poor prognostic factors include age < 14 yo or syncope at presentation.
Also FH of HCM or sudden death