Hypertrophic Cardiomyopathy A Contemporary Treatable Disease

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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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