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Definition epidemiology pathology of hypertrophic cardiomyopathy

  • LV (usually ≥15 mm) and/or RV hypertrophy disproportionate to hemodynamic load
  • Due to gene mutations affecting proteins of or related to sarcomere; prev.: ~1/200-500
  • Myocardial fiber disarray with hypertrophy, which creates arrhythmogenic substrate
  • Many morphologic hypertrophy variants: asymmetric septal; concentric; midcavity; apical
  • Ddx: LVH 2° to HTN, AS, elite at ↣ hletes (wall usually <13 mm & symmetric and nl/↑ rates of tissue Doppler diastolic relaxation; Circ 2011;123:2723), Fabry dis. (↑ Cr, skin findings)