Hypertrophic Cardiomyopathy Flashcards

1
Q

What type of condition is Hypertrophic cardiomyopathy?

A

Autosomal dominant genetic disorder

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2
Q

What does hypertrophic cardiomyopathy cause?

A

Thickening of the myocardium, of the left ventricle

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3
Q

What is hypertrophic cardiomyopathy due to?

A

A mutation in either
–the gene encoding β-myosin heavy chain protein
–myosin-binding protein C

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4
Q

What is hypertrophic cardiomyopathy a leading cause of?

A

Leading cause of sudden cardiac death in young athletes

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5
Q

What does hypertrophy of the left ventricle result in?

A
  • left ventricular outflow obstruction
  • mitral valve problems
  • myocardial ischaemia
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6
Q

Does hypertrophic cardiomyopathy cause systolic or diastolic dysfunction?

A

Diastolic dysfunction

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7
Q

How can hypertrophic cardiomyopathy present?

A
  • Dyspnoea
  • Chest pain
  • Exertional Syncope (on exercise)
  • Palpitations
  • Mitral regurgitation
  • Jerky pulse
  • Ejection systolic murmur
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8
Q

How does hypertrophic cardiomyopathy usually present?

A

Asymptomatically

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9
Q

What is sudden cardiac death due to in people with hypertrophic cardiomyopathy?

A

ventricular arrhythmia

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10
Q

What affects the ejection systolic murmur in someone with hypertrophic cardiomyopathy?

A

Increases with Valsalva manoeuvre
Decreases on squatting

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11
Q

Why might someone with hypertrophic cardiomyopathy present with mitral regurgitation?

A

Hypertrophic cardiomyopathy may impair mitral valve closure

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12
Q

What is hypertrophic cardiomyopathy associated with?

A
  • Friedreich’s ataxia
  • Wolff-Parkinson White
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13
Q

How is hypertrophic cardiomyopathy diagnosed?

A

Echocardiogram

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14
Q

What might you find on an echocardiogram of someone with hypertrophic cardiomyopathy?

A

Mr SAM ASH
* Mitral Regurgitation (MR)
* Systolic anterior motion (SAM) of the anterior mitral valve
* Asymmetric septal hypertrophy (ASH)

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15
Q

What ECG changes may be seen in someone with hypertrophic cardiomyopathy?

A
  • left ventricular hypertrophy
  • T-wave abnormalities- progressive T wave inversion
  • deep Q waves (dagger q waves)
  • atrial fibrillation
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16
Q

What might be seen on biopsy of someone with hypertrophic cardiomyopathy?

A

Myofibrillar hypertrophy with chaotic and disorganized fashion myocytes (‘disarray’) and fibrosis

17
Q

What is the management of an asymptomatic patient with hypertrophic cardiomyopathy?

A

Do nothing

18
Q

What is the first line management of a patient with hypertrophic cardiomyopathy?

A

Amiodarone

19
Q

What is the function of amiodarone in someone with hypertrophic cardiomyopathy?

A

Control arrhythmias

20
Q

What other medication would you give someone with hypertrophic cardiomyopathy?

A
  • Beta-blockers or verapamil
21
Q

What is the function of Beta-blockers or verapamil?

A

Reduce LVOT obstruction

22
Q

What medication should be avoided in someone with HoCM?

A

ACEi

23
Q

What additional heart sound is HOCM associated with?

A

S4

24
Q

If one sibling has HOCM, what is the chance that the other sibling will have it?

A

50% chance, no carrier state