its also called Asymmetric Septal Hypertrophy and also some books called it Idiopathic Hypertrophic Subaortic stenosis.
so from the name its characterized by hypertrophy specially if the left ventricle, the ventricle wall becomes thick, stiff, and thick, stiff and solid, and for this reason there will be forceful contraction that rapidly expel or push the blood, but there will be problems in diastole, there will be abnormal diastolic filling, there is no relaxation in the ventricle , the blood in the ventricle is greatly reduced, the so the ejection or expelling from the ventricle is ineffective (amount expelled is low), is in general what we see is decrease in cardiac output.
Pathogenesis:
studies said that 50% of HCM cases is inherited autosomal dominant trait and usually occurred by either:
pathology:
because of the hypertrophy, the heart become big and will wiegh more than 800gm. we see hypertrophy in the left ventricle and inter-ventricular spetum without dilation, the dilation may occur in the left atrium.
thickening in the walls of the ventricle will not be the same, for that reason it will be called Asymmetric septal hypertrophy.
so from the name its characterized by hypertrophy specially if the left ventricle, the ventricle wall becomes thick, stiff, and thick, stiff and solid, and for this reason there will be forceful contraction that rapidly expel or push the blood, but there will be problems in diastole, there will be abnormal diastolic filling, there is no relaxation in the ventricle , the blood in the ventricle is greatly reduced, the so the ejection or expelling from the ventricle is ineffective (amount expelled is low), is in general what we see is decrease in cardiac output.
Pathogenesis:
studies said that 50% of HCM cases is inherited autosomal dominant trait and usually occurred by either:
- mutation in gene that synthesize the proteins such as beta-myosin heavy chain which occurs in about 30% of cases. \
- troponin I and T, alpha-tropomyosin and myosin ligh chains.
- allelic heterogeneity which mean that genotype is caused by different mechanism.
pathology:
because of the hypertrophy, the heart become big and will wiegh more than 800gm. we see hypertrophy in the left ventricle and inter-ventricular spetum without dilation, the dilation may occur in the left atrium.
thickening in the walls of the ventricle will not be the same, for that reason it will be called Asymmetric septal hypertrophy.
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