Hypertrophic Cardiomyopathy Flashcards
What type of condition is Hypertrophic cardiomyopathy?
Autosomal dominant genetic disorder
What does hypertrophic cardiomyopathy cause?
Thickening of the myocardium, of the left ventricle
What is hypertrophic cardiomyopathy due to?
A mutation in either
–the gene encoding β-myosin heavy chain protein
–myosin-binding protein C
What is hypertrophic cardiomyopathy a leading cause of?
Leading cause of sudden cardiac death in young athletes
What does hypertrophy of the left ventricle result in?
- left ventricular outflow obstruction
- mitral valve problems
- myocardial ischaemia
Does hypertrophic cardiomyopathy cause systolic or diastolic dysfunction?
Diastolic dysfunction
How can hypertrophic cardiomyopathy present?
- Dyspnoea
- Chest pain
- Exertional Syncope (on exercise)
- Palpitations
- Mitral regurgitation
- Jerky pulse
- Ejection systolic murmur
How does hypertrophic cardiomyopathy usually present?
Asymptomatically
What is sudden cardiac death due to in people with hypertrophic cardiomyopathy?
ventricular arrhythmia
What affects the ejection systolic murmur in someone with hypertrophic cardiomyopathy?
Increases with Valsalva manoeuvre
Decreases on squatting
Why might someone with hypertrophic cardiomyopathy present with mitral regurgitation?
Hypertrophic cardiomyopathy may impair mitral valve closure
What is hypertrophic cardiomyopathy associated with?
- Friedreich’s ataxia
- Wolff-Parkinson White
How is hypertrophic cardiomyopathy diagnosed?
Echocardiogram
What might you find on an echocardiogram of someone with hypertrophic cardiomyopathy?
Mr SAM ASH
* Mitral Regurgitation (MR)
* Systolic anterior motion (SAM) of the anterior mitral valve
* Asymmetric septal hypertrophy (ASH)
What ECG changes may be seen in someone with hypertrophic cardiomyopathy?
- left ventricular hypertrophy
- T-wave abnormalities- progressive T wave inversion
- deep Q waves (dagger q waves)
- atrial fibrillation
What might be seen on biopsy of someone with hypertrophic cardiomyopathy?
Myofibrillar hypertrophy with chaotic and disorganized fashion myocytes (‘disarray’) and fibrosis
What is the management of an asymptomatic patient with hypertrophic cardiomyopathy?
Do nothing
What is the first line management of a patient with hypertrophic cardiomyopathy?
Amiodarone
What is the function of amiodarone in someone with hypertrophic cardiomyopathy?
Control arrhythmias
What other medication would you give someone with hypertrophic cardiomyopathy?
- Beta-blockers or verapamil
What is the function of Beta-blockers or verapamil?
Reduce LVOT obstruction
What medication should be avoided in someone with HoCM?
ACEi
What additional heart sound is HOCM associated with?
S4
If one sibling has HOCM, what is the chance that the other sibling will have it?
50% chance, no carrier state
