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Idiopathic Hypertrophic Subaortic Stenosis
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Hypertrophic
cardiomyopathy
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- 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.
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- Shortness of breath
- Chest pain
- Palpitations
- Fainting (especially after
exertion)
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- 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.
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- Beta-blockers
- 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
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- Atrial fibrillation -- a heart
arrhythmia
- Sudden
death
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- 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.
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