Hypertrophic cardiomyopathy: presentation and sudden death
THE CONTEXT
Effort-related symptoms dominate the clinical presentation, and sudden death is a major concern during vigorous physical activity.
Clinical presentation
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Dominant presentations: Angina, breathlessness, arrhythmia and sudden death are effort-related presentations.
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Arterial pulse: Symptoms and signs resemble aortic stenosis, but the arterial pulse in hypertrophic cardiomyopathy is jerky.
Sudden death and natural history
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Adult mortality: Annual mortality from sudden death is 2%–3% among adults.
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Younger patients: Annual mortality from sudden death is 4%–6% in children and adolescents.
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Timing and mechanism: Sudden death typically occurs during or just after vigorous physical activity and is thought to result from ventricular arrhythmias.
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Young athletes: Hypertrophic cardiomyopathy is the most common cause of sudden death in young athletes.
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Clinical course: In patients without fatal arrhythmias, the natural history varies, but clinical deterioration is often slow.