Key result
Ventricular arrhythmias and sudden death in hypertrophic cardiomyopathy are driven by substrates including hypertrophy, fibrosis, and abnormal gap junction distribution predisposing to re-entry.
This review highlights the pathophysiological substrates—such as hypertrophy, fibrosis, and gap junction abnormalities—that predispose patients with hypertrophic cardiomyopathy to life-threatening ventricular arrhythmias and sudden death.
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Reinforces substrate-focused risk models in HCM; leaves open targeted anti-fibrotic or gap-junction therapies.
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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Michiel J. Janse (2001) conducted a review in Hypertrophic cardiomyopathy. Ventricular arrhythmias and sudden death in hypertrophic cardiomyopathy are driven by substrates including hypertrophy, fibrosis, and abnormal gap junction distribution predisposing to re-entry.
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