Hypertrophic Obstructive Cardiomyopathy

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

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.