(Adapted from ESC/EHA Guidelines)

  • Classically associate with a prolonged asymptomatic period followed by rapid progression of symptoms
    • Asymptomatic disease:
      • Risk of sudden death of 1%
    • Symptomatic disease:
      • Poor prognosis without valve replacement
      • Average survival 2-5 years
      • 50% incidence of sudden death and a monthly mortality of 2%
  • There is no definitive evidence that medical treatment alone can slow disease progression