Sudden death in patients without structural heart disease

Sudden unexpected cardiac death generally occurs in persons with known or previously unrecognized heart disease. However, it has become evident that it occurs often enough in patients without any identifiable structural abnormality to warrant the cardiologist's attention. Mostly, it concerns young, active, and otherwise healthy individuals. This paper focuses on various categories of patients with life-threatening events considered to have occurred on a solely "electrical" basis. Currently, several entities are recognized with distinct electrophysiological abnormalities, including Wolff-Parkinson-White syndrome, long QT syndrome, the Brugada syndrome, short-coupled torsade de pointes, and catecholamine-induced polymorphic ventricular tachyarrhythmia. The remaining patients without such distinct abnormalities are categorized as having idiopathic ventricular fibrillation. Although mechanical cardiac function may seem normal, such patients might have certain discrete anatomic abnormalities, unidentifiable with current investigational tools. Possibly in the future, with development of newer and more sophisticated tools (magnetic resonance imaging, positron emission tomography, genetic testing), some or all cases of idiopathic ventricular fibrillation must be redefined as having specific genetic and/or anatomic bases. All patients successfully resuscitated from cardiac arrest due to ventricular tachyarrhythmia without clear precipitating factors (acute myocardial infarction, severe electrolyte or metabolic disturbances) are at high risk of recurrences. Long-term prophylactic therapy is indicated. Contrasting with older belief, survivors of idiopathic ventricular fibrillation are now also considered high-risk patients. The implantable cardioverter-defibrillator appears to be the safest and most effective therapy.