1. LVH & Heart murmur Murmur Increased w/ standing
Decreased w/ supine leg raising, squatting
Markedly increased with Amyl Nitrite*
*Don’t try this at home

2. Cardiomyopathy Cardiomyopathy Hypertrophic cardiomyopathy (HC)
Restrictive
Dilated
Hypertrophic
Hypertrophic cardiomyopathy (HC)
AKA:
Idiopathic Hypertrophic Subaortic IHSS
ASH
Dilated <40%    Impairment of contractility (systolic dysfunction)      Idiopathic; alcohol; peripartum; genetic; myocarditis; hemochromatosis; chronic anemia; doxorubicin (Adriamycin); sarcoidosis   Ischemic heart disease; valvular heart disease; hypertensive heart disease; congenital heart disease    Hypertrophic    50-80%  Impairment of compliance (diastolic dysfunction)        Idiopathic; genetic; Friedreich ataxia; storage diseases; infants of diabetic mothers   Hypertensive heart disease; aortic stenosis     Restrictive     45-90%  Impairment of compliance (diastolic dysfunction)        Idiopathic; amyloidosis; radiation-induced fibrosis     Pericardial constriction        *Normal, approximately 50-65%.

3. 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

4. Hypertrophic cardiomyopathy
Symptoms
None –> sudden death
Implicated in sudden death of athletes
Dyspnea, palpatations, angina, syncope

5. 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

6. EKG
Pseudoinfarct patterns with noninfarctional Q waves and ST-T-wave changes
“Differentiating the EKG pseudoinfarctional patterns of IHSS from AMI may be impossible. “
Hypertrophic cardiomyopathy is an important cause of pseudoinfarct patterns and may be associated with noninfarctional Q waves and ST-T-wave changes in any of the standard leads, thereby resembling acute or evolving AMI.
Patients with apical hypertrophic cardiomyopathy commonly have characteristic deep precordial T-wave inversions, sometimes exceeding 10 mm in size. [213 <12210.html>]
The pathogenesis of these pseudoinfarct patterns may result from the anatomic location and mass of myocardial hypertrophy, which dictates the morphology and lead location of noninfarctional Q waves. For example, the initial forces of septal depolarization spread from left to right, initially directed away from the left precordial leads. Massive septal hypertrophy accounts for the deep Q waves that may appear in the lateral or inferior leads and that resemble inferior or anterolateral AMI.

7. Evaluation Echo Thalium (may show defects w/ nl coronary arteries)
Cardiac cath

8. Treatment Reduce contractility, increase ventricular volume
Beta blockers, Calcium channel blockers
Avoid sports or strenuous activity

9. 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