Interesting Cases

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LVH Heart murmur

• Murmur
• Increased w/ standing
• Decreased w/ supine leg raising, squatting
• Markedly increased with Amyl Nitrite
• Dont try this at home

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Cardiomyopathy

• Cardiomyopathy
• Restrictive
• Dilated
• Hypertrophic
• Hypertrophic cardiomyopathy (HC)
• AKA
• Idiopathic Hypertrophic Subaortic IHSS
• ASH

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Hypertrophic cardiomyopathy (HC)

• Asymmetric abnormal septal growth associated with
  subaortic obstruction.
• Dynamic obstruction
• Increases w/ decreased ventricle size
• Often presents in 3rd decade but can range from
  infants elderly
• Etiology ?
• Autosomal Dominant

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Hypertrophic cardiomyopathy

• Symptoms
• None gt sudden death
• Implicated in sudden death of athletes
• Dyspnea, palpatations, angina, syncope

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Exam

• Can be normal
• Signs LVH
• Murmur
• Harsh crescendo decrescendo systolic murmur at
  the apex
• Increased w/ standing, tachycardia, Amyl Nitrite,
  NTG, exercise
• Decreased w/ squatting, isometric hand grip, beta
  blockade

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EKG

• Pseudoinfarct patterns with noninfarctional Q
  waves and ST-T-wave changes
• Differentiating the EKG pseudoinfarctional
  patterns of IHSS from AMI may be impossible.

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Evaluation

• Echo
• Thalium (may show defects w/ nl coronary
  arteries)
• Cardiac cath

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Treatment

• Reduce contractility, increase ventricular volume
• Beta blockers, Calcium channel blockers
• Avoid sports or strenuous activity

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Why is this important for us?

• Sudden death in a young person is bad
• Difficult to predict
• Syncope is ominous in kids, not a predictor in
  adults
• HX NSVT is a predictor in adults
• Relatives should be evaluated
• Avoid certain drugs
• Difficult to differentiate from normal
  hypertrophy seen in athletes