Abstract

Ventricular arrhythmias and sudden death are among the characteristics of hypertrophic cardiomyopathy. Experience with the implantable cardioverter-defibrillator has shown that sudden death is caused by ventricular tachycardia or fibrillation. Several triggers for sudden death have been proposed: arrhythmias; ischaemia; and abnormal response to exercise. The substrates for arrhythmias includes hypertrophy itself, predisposing to triggered activity based on delayed afterdepolarizations; fibrosis, leading to abnormal, zig-zag conduction and fractionated extracellular electrograms; and abnormal distribution of gap junctions, especially in areas of myofibrillar disarray, which also may lead to conduction abnormalities. The latter two factors predispose to re-entry.

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