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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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