Hypertrophic Cardiomyopathy

on 23.1.08 with 0 comments



Synonyms:
  • symmetric septal hypertrophy
  • idiopathic hypertrophic subaortic stenosis
  • hypertrophic subaortic stenosis
Imp Points:
  • AD (50%)
  • Inherited Sarcomeric mutations
  • 1/3 caused by mutation of β myosin heavy chain. Also troponin and tropomyosin
  • Most common reason for heart deaths in young athletes
  • Young men
Pathological/Clinical Picture:
  • Hypertrophy of all chamber walls, especially IV septum
  • Casues diastolic failure (stiff ventricle can’t fill)Disoriented, tangled, hypertrophied fibers
  • Causes LV outflow obstruction (30%), esp if asymmetric IV septum hypertrophy just below the aorta
    • Causes ant leaflet of mitral valve to hit IV septum → thickening of endocardium on leaflet and septal wall
  • Causes ↑ in pulm venous P and dilated LA
  • Late stage fibrosis → CHF
  • Harsh systolic ejection murmur
  • Myocardial ischemia and ventricular arrythmia
Mimicked by amyloidosis, glycogen storage disease

Category: Pathology Notes

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