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Idiopathic Hypertrophic Subaortic Stenosis

more about Idiopathic Hypertrophic Subaortic Stenosis

Hypertrophic cardiomyopathy

  • The heart has four chambers to pump blood throughout the body. In Hypertrophic Cardiomyopathy, the septal portion of the heart becomes enlarged and thickened.
  • Septal enlargement (hypertrophy) takes up part of the heart chamber cavity, creating an obstruction to the flow of blood, especially from the left ventricle.

  • Shortness of breath
  • Chest pain
  • Palpitations
  • Fainting (especially after exertion)

  • Heart exam revealing a loud S4.
  • Murmur that worsens with Valsalva's maneuver and decreases with squatting.
  • Electrocardiogram shows left ventricular hypertrophy
  • Chest X-Ray is usually normal
  • Echocardiogram shows asymmetrical left ventricular hypertrophy and delayed filling of the left ventricle. Doppler test shows turbulent flow and dynamic gradient across the aortic valve, along with mitral regurgitation.

  • Medications:
    1. Beta-blockers
    2. Negative inotropic Calcium channel blockers such as Verapamil
  • Surgical excision of the part of the myocardial septum in severe cases
  • Dual chamber pacemakers are helpful in decreasing progression of hypertrophy
  • Sotalol or Amiodarone may be used to prevent arrythmias

  • Atrial fibrillation -- a heart arrhythmia
  • Sudden death

  • Since the disease progresses without treatment, you need to seek medical treatment as soon as possible. This disease also can result in sudden death, especially after exertion (e.g., exercise), so diagnosing and optimizing treatment is important.

more about Idiopathic Hypertrophic Subaortic Stenosis

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