Genetic defect in chromosome 1, 11, 14, or 15; encoding of pathological myofibrils
ECG characteristics:
Often highly positive Sokolow index (see 5.1 Left Ventricular Hypertrophy)
Marked T inversion in the precordial leads (pseudo-infarction ECG)
Mechanism:
Hypertrophy-related relative ischemia of the inner layer causing disturbance of repolarization in this region, flow of current from outer to inner layer
Obstruction:
LVOT, apical, midseptal
No obstruction (HNCM)
Treatment:
Beta-blocker, calcium antagonists (verapamil)
Pacemaker
Interventional sclerosis of septal branch (TASH), operative intervention (myectomy)
Arrhythmia prophylaxis (amiodarone)
Insertion of debrillator with occurrence of malignant ventricular arrhythmias
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