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Hypertrophic Obstructive Cardiomyopathy (HOCM)
- outflow obstruction in 1/3 cases of hypertrophic cardiomyopathy
- inherited disorder of heart muscle
- variable hypertrophy of left and/or right ventricle without systemic cause
- massively thickened interventricular septum (characteristic but not
inevitable)
- distorted ventricular contraction
- abnormal mitral valve movement
- mitral regurgitation
- apposition of anterior mitral leaflet to interventricular septum causes
obstruction of left ventricular emptying
Pathology
- characteristic myocytes disarray
Inheritance
- familial, autosomal dominant
- beta myosin
- troponin T
- alpha tropomyosin
- myosin binding protein C
Complications
- risk of sudden death
- increased DD alleles of ACE associated with sudden death
Associations
History
- chest pain
- dyspnoea
- due to inability of cardiac muscle to relax
- syncope / pre-syncope
- cardiac arrythmias
- sudden death
- asymptomatic adolescents / young adults
Examination
- double apical pulsation
- jerky carotid pulse
- ejection systolic murmur
- left ventricular outflow obstruction
- pansystolic murmur
- fourth Heart Sound
Investigations
- CXR
- ECG
- left ventricular hypertrophy
- ST wave / T wave changes
- Echocardiogram
- left ventricular hypertrophy
- systolic anterior motion of mitral valve
- very vigorous contraction of ventricle
- Excercise tolerance test
- Ambulatory ECG
Treatment
- Avoidance of sudden death
- amiodarone
- implantable defibrillator
- chest pain / dyspnoea
- alcohol ablation of septum
- avoid vasodilators
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