Hypertrophic Cardiomyopathy Flashcards

1
Q

What is hypertrophic cardiomyopathy (HCM)?

A

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.

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2
Q

Clinical features of HCM.

A

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

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3
Q

Investigations of HCM.

A

ECG

Echo

MRI

Cardiac catheterisation

Exercise test +/- holter monitoring

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4
Q

ECG findings in HCM.

A

LVH

Progressive T-wave inversion

Deep Q waves in inferior and lateral leads.

AF

WPW syndrome

Ventricular ectopics

VT

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5
Q

Echo findings in HCM.

A

Asymmetrical septal hypertrophy

Small LV cavity

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6
Q

Why might a cardiac catherisation be done?

A

To assess severity of disease.

However it can cause VT

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7
Q

Management of HCM.

A

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

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8
Q

Prognosis of HCM.

A
  1. 9%/yr if < 14 yo at presentation
  2. 5%/yr if > 14 yo

Poor prognostic factors include age < 14 yo or syncope at presentation.

Also FH of HCM or sudden death

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