Hypertrophic Cardiomyopathy A Contemporary Treatable …
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Hypertrophic Cardiomyopathy A Contemporary Treatable Disease
Dr. Grant L. Peters September 27, 2018
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? First description of asymmetric hypertrophy in 1869 ? 2 French physicians (Liouville and Hallopeau)
? Despite its initial description ? not studied systematically until 1950's
? 1958 ? Donald Teare ? reported the pathologic findings ? 8 young patient (7 died suddenly)
? Massive hypertrophy septum with small LV cavity size ? Microscopically ? myocardial disarray ? Etiology unclear ? (? Benign tumor or Hamartoma)
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? 1950's ? Sir Russell Brock ? reported on patients with functional subvalvular LVOT gradients ? previously diagnosed as valvular aortic stenosis
? 1960's ? Braunwald et al ? defined the disease process
? Asymmetric septal hypertrophy ? Myofibril disarray ? Dynamic subvalvular pressure gradient
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? Described by many names (>75)
? IHSS ? Idiopathic Hypertrophic Subaortic Stenosis ? ASH ? Asymmetric Septal Hypertrophy ? HOCM ? Hypertrophic Obstructive CM ? Muscular subaortic stenosis
? Minority demonstrate outflow gradient
? WHO ? Hypertrophic CM ? primary muscular hypertrophy with or without obstruction
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? Emergence of new diagnostic tools (2D and M mode echocardiography) ? new understanding
? More common ? Causes of obstruction ? Variable distribution and extent of hypertrophy ? Hemodynamics
? Recognized disease ran in families
? 1980's genetic nature of the disease ? 1989 ? linked to gene on chromosome 14
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? Primary disease of the cardiac sarcomere
? Mutations of proteins
? Beta myosin ? Troponin I ? Troponin T ? -tropomyosin ? Myosin Protein C ? Myosin light chain ? Alpha actin ? Titin
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? Genetic disease affecting the cardiac sarcomere ? autosomal dominant pattern of inheritance
? Presence of left ventricular hypertrophy ( 15 mm) in whom the degree of hypertrophy is not explained by another cardiac cause or systemic disease
? Most common causes of hypertrophy are HTN, aortic stenosis, athletic heart
? 13 mm if known family history of HCM
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