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Common Congenital Heart Lesions - page 96 / 126

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96 / 126

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Hypertrophic Cardiomyopathy (HCM)

  • Most common cardiac abnormality found in young athletes who die suddenly during vigorous physical exertion.

  • Septal or LV hypertrophy that is NOT due to chronic pressure overload

  • A.k.a Æ hypertrophic obstructive cardiomyopathy (HOCM) and idiopathic hypertrophic subaortic stenosis (IHSS)

ETIOLOGY

  • Familial disease… (genetic)… autosomal dominant

  • Mutation causes impaired contractile fxn Æ incr myocyte stress Æ compensatory hypertrophy and proliferation of fibroblasts

  • Precise mutation determines age of onset of hypertrophy, extent and patern of cardiac remodeling, and individual’s risk of developing symptomatic heart failure or sudden death

PATHOLOGY

  • Hypertrophy of any portion of the ventricles, but asymmetric hypertrophy of the ventricular septum is most common

  • Histology is unusual and doesn’t reflect normal hypertrophy of myocytes… myocyte disarray and fibrosis = diagnostic of HCM and play role in stiffness and arrhythmias

PATHOPHYS

  • Predominant feature = ventricular hypertrophy that reduces compliance and relaxation (diastolic fxn) of the chamber such that filling becomes impaired

  • Myocyte disarray Æ arrhythmias

  • Can have HCM w/ or w/out an outflow tract obstruction depending on where hypertrophy is and how bad it is:

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