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Transcript
Hypertrophic Cardiomyopathy Lange-pp. 362-364 http://www.mayoclinic.com/health/hypertrophic-cardiomyopathy/DS00948 http://www.4hcm.org/WCMS/index.php Essentials of Diagnosis May present with dyspnea, chest pain, syncope. Though LV outflow gradient is classic, symptoms are primarily related to diastolic dysfunction Echocardiogram shows septal hypertrophy, which is usually asymmetric, and enhanced contractility. Systolic anterior motion of the anterior mitral valve is present if there is outflow tract obstruction. The highest risk group for sudden death includes those with a marked LVH, with a family history for sudden death, with ventricular ectopy, and with an abnormal BP response to exercise. Genetic disease with prevalence of 1 in 500 in the general adult population. Caused by mutations in any of 10 genes coding for proteins of the cardiac sarcomere. Genetic screening is currently limited to screening known or suspected familial groups Hallmark abnormality of HCM is: 1. Inappropriate and marked left ventricular hypertrophy, ranging from mild to extreme, esp. localized hypertrophy, typically in the anterior septum. This hypertrophy may result in areas of ischemia or infarction due to abnormal microvasculature, impaired coronary vasodilator reserve, or mismatch between myocardial mass and coronary circulation. This causes scarring. 2. Outflow tract obstruction, not demonstrated in the majority; causes a loud systolic murmur and may be symptomatic with exertion intolerance, syncope, or sudden death Used to be called HOCM (hypertrophic obstructive cardiomyopathy) or IHSS (idiopathic hypertrophic subaortic stenosis) Male predominance slightly up to mid life; female predominance is found in patients older thatn 60 Presenting signs and symptoms: Majority of patients are asymptomatic or have mild symptoms Dyspnea, angina, presyncope or syncope with symptoms worse with exertion. Atrial fibrillation occurs in ¼ of patients thus hemodynamic compromise because of inadequate filling of the hypertrophied ventricle. Physical examination: Forceful and enlarged precordial impulse, often laterally displaced Murmur results from flow through the left ventricular outflow tract, and is usually a harsh crescendo-decrescendo, starts well after the first heart sound and is best heard between the apex and left sterna border. The murmur increases with maneuvers that increase the gradient through increased contractility, decreased preload, or decreased afterload; conversely, the murmur decreases with interventions that decrease contractility, increase preload or increase afterload. Squatting (increased afterload) = decreased murmur Standing (decreased afterload) = increased murmur Valsalva (decreased preload) = increased murmur Precordial impulse-presystolic, systolic, and late systolic impulse (“triple ripple”) Syncope, history of cardiac arrest, documented sustained ventricular tachyarrhythmias and family history of sudden death are predictors of sudden death. The ECHO is essential and helps to examine outflow tract obstruction EKG-left ventricular hypertrophy and prominent Q waves in the inferior and lateral leads due to septal hypertrophy. Giant negative T waves suggest the apical variant of HCM Stress testing Beta blockers are considered the initial therapy of choice; AICD if considered high risk for sudden death