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12 Cards in this Set
- Front
- Back
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definition of hypertrophic cardiomyopathy
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inappropriate asymmetric ventricular hypertrophy without cardiac or systemic cause
autosomal dominant - mutation in sarcomere contractile protein most common cause of sudden death in young people - especially athletes |
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hypertrophic cardiomyopathy pathophysiology
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mutation in sarcomere contractile protein - most common beta-myosin heavy chain Chr14
myocyte hypertrophy and disarray -> outflow track blocking and arrhythmias usually a subaortic dilation, put possible to have midventricular, apical, diffuse forms septum gets so big |
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how does hypertrophic cardiomyopathy cause angina
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1. wall is too thick, demands more O2 and nutrients
2. outflow obstruction means heart must pump harder -> increased O2/nutrient requirements |
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how does hypertrophic cardiomyopathy cause syncope
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1. outflow obstruction - decreased CO
2. Vasodepressor reflex - due to high pressure in ventricle the body reacts by decreasing peripheral resistance -> drop in BP |
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how does hypertrophic cardiomyopathy cause dyspnea
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LV can't pump properly and often see mitral regurgitation -> LA works harder -> back up of blood into pulmonary
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hypertrophic cardiomyopathy murmur
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gradient between the LV and aorta creates a systolic murmur
anything that increases gradient will make murmur worse decreasing preload/afterload (nitrites, standing, valsalva) or increases in contractility - make gradient/murmur worse increasing preload/afterload (squatting, leg raising) - makes gradient smaller/ murmur better |
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what other murmur reacts in a similar pattern to hypertrophic cardiomyopathy in regards to increased or decreased preload/afterload?
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mitral prolapse murmur
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hypertrophic cardiomyopathy left ventricular outflow tract obstruction
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thickened septal wall can cause obstruction below the aortic valve (subaortic)
pressure gradient sucks in anterior leaflet of mitral valve to create obstruction worsened by decreased preload, decreased afterload, or increased contraction |
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what do you see on EEG for hypertrophic cardiomyopathy?
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inverted T waves - not diagnostic, nonspecific
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poor prognostic factors of hypertrophic cardiomyopathy
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younger age
male positive family history history of syncope severe hypertrophy on echo genetic markers |
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arrhythemias in hypertrophic cardiomayopathy
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arterial arrhythemias are common -> hemodynamic deterioration
afib is really bad - HCM patient relies on atrial function for LV filling - afib -> huge decrease in CO VT/VF -> sudden death |
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management of hypertrophic cardiomyopathy
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screen family
genetic counsling for younger avoid athletics, strenuous activity, dehydration holter monitor to watch for VT/VF drugs: first line is beta blocker treatment: septal myotomy (gold standard), or alcohol ablation |
