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

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%.

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

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

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

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.

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

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

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