Hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is an autosomal dominant cardiac condition characterized by marked left ventricular hypertrophy with a non-dilated ventricular cavity, and in around 20% of patients left ventricular outflow obstruction

It is the commonest genetic cardiac disease (incidence 1 in 500) and the commonest cause of sudden cardiac death in the young. 

Presentation may be: 
  • Sudden death 
  • Syncope 
  • Shortness of breath 
  • Angina 
  • Palpitations 

Clinical findings may include: 
  • Ejection systolic murmur 
    • If obstruction is present this murmur may be increased by valsalva 
  • Prominent A wave in JVP
  • Bifid pulse (obstructive only) 
  • ? MR 
  • ? split S2 

  • Echocardiography 
    • Hypertrophied and non-dilated left ventricle 
  • ECG
    • ? LVH 
    • ? AF 
    • ? Q waves 
    • ? TWI 

Prognosis include:
  • Sudden death (classically stated at 6% per year but now believed to be lower – around 1%) 
  • Atrial fibrillation (20-25%) 
  • Heart failure symptoms 
  • No symptoms 

Risk factors for sudden death 
  • Family history of sudden death 
  • Unexplained syncope 
  • Massive LV hypertrophy (thickness >30mm)
  • Non-sustained VT on ambulatory ECG 
  • Hypotensive on exercise 

Management – depends on severity and symptoms 
  • Medical 
    • Beta blockers 
    • Verapamil 
  • Surgery 
  • Percutaneous alcohol septal ablation 
  • Dual chamber pacing
  • ICD 
  • Avoid nitrates/ACE-i/digoxin in outflow tract obstruction 

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Secret collector of interesting anonymised ECGs. Fan of the Bath Photomarathon. Lover of cream teas. [Sarah Hudson] (Your Picture)