Hypertrophic Obstructive Cardiomyopathy( HOCM)idiopathic hypertrophic subaortic stenosis( IHSS)asymmetric septal hypertrophy
AD disease in 50% with defects in genes for troponin, tropomyosin and myosin
Hypertrophy of septum more than ventricular wall causing diastolic dysfunction and obstructionof outflow, with hyperkinetic contraction and LA dilation
Morphology:
M/E haphazard array of hypertrophied abnormally branching myocytes with connective tissue deposition
C/F
Exertional dyspnea, angina, CHF
Arythmia causing sudden cardiac death
ejection systolic murmur in aortic area radiating to carotids
Increased risk of infective endocarditis
Hypertrophic cardiomyopathy – myofiber dysarray – not all fibers are pulling
the same direction. Thus the contraction is ineffective. However, the cardiac
conduction system can have these same problems, which might cause the arrhythmias and sudden death these patients tend to die of.
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