Station 3.12: Hypertrophic (obstructive) cardiomyopathy

Question 1

Clinical signs

What are the clinical signs for Hypertrophic (obstructive) cardiomyopathy?

Hypertrophic (obstructive) cardiomyopathy

This young man has complained of palpitations whilst playing football. Examine his cardiovascular system.

Answer 1

• Jerky pulse character
• Double apical impulse (palpable atrial and ventricular contraction)
• Thrill at the lower left sternal edge
• Auscultation:

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• There may be associated mitral valve prolapse (MVP) (see page 52)
• There may be features of Friedreich’s ataxia or myotonic dystrophy (see Neurology section)

Question 2

Discussion - investigation

What are the clinical investigations for Hypertrophic (obstructive) cardiomyopathy?

Hypertrophic (obstructive) cardiomyopathy

This young man has complained of palpitations whilst playing football. Examine his cardiovascular system.

Answer 2

• ECG: LVH with strain (deep T‐wave inversion across precordial leads)
• CXR: often normal
• TTE: asymmetrical septal hypertrophy and systolic anterior motion of the anterior mitral leaflet across the LVOT due to misalignment of septal papillary muscle, LVOT gradient (rest/exercise or dobutamine stress)
• Cardiac MR: identifies apical HCM more reliably than TTE
• Cardiac catheterization: gradient accentuated by a ventricular ectopic or pharmacological stress, identification of septals
• Genetic tests: sarcomeric proteins mutation

Question 3

Discussion - Management

What are the clinical Managements for Hypertrophic (obstructive) cardiomyopathy?

Hypertrophic (obstructive) cardiomyopathy

This young man has complained of palpitations whilst playing football. Examine his cardiovascular system.

Answer 3

Asymptomatic:
* Avoidance of strenuous exercise, dehydration and vasodilators

Symptomatic and LVOT gradient >30mm Hg
* β‐Blockers
* Pacemaker
* Alcohol septal ablation
* Surgical myomectomy

Rhythm disturbance/high‐risk SCD
* ICD

Refractory:
* Cardiac transplant

Genetic counselling of first‐degree relatives (autosomal dominant inheritance)

Question 4

Discussion - Prognosis

What is the Prognosis for Hypertrophic (obstructive) cardiomyopathy?

Hypertrophic (obstructive) cardiomyopathy

This young man has complained of palpitations whilst playing football. Examine his cardiovascular system.

Answer 4

• Annual mortality rate in adults is 2.5%
• Poor prognosis factors:
  ⚬ Young age at diagnosis
  ⚬ Syncope
  ⚬ Family history of sudden death
  ⚬ Septal thickness > 3cm