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STATE-OF-THE-ART PAPER

Sudden death in patients without structural heart disease

Eric F. D. Wever, MD, PhD^*,* and Etienne O. Robles de Medina, MD, PhD, FACC

^* Department of Cardiology, Heart Lung Center, Utrecht, The Netherlands
St. Antonius Hospital, Nieuwegein, The Netherlands
University Medical Center Utrecht, Utrecht, The Netherlands

Manuscript received December 4, 2002; revised manuscript received October 7, 2003, accepted October 30, 2003.

^* Reprint requests and correspondence: Dr. Eric F. D. Wever, Department of Cardiology, St. Antonius Hospital, Koekoekslaan 1/PO-Box 2500, 3435 CM/3430 EM, Nieuwegein, The Netherlands.
e.wever{at}antonius.net

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.

Abbreviations and Acronyms   AV = atrioventricular   EPS = electrophysiologic study   ICD = implantable cardioverter-defibrillator   IVF-US = Idiopathic Ventricular Fibrillation Registry of the United States of America   LQTS = long QT syndrome(s)   SCD = sudden cardiac death   U-CARE = Unexplained Cardiac Arrest Registry Europe   VF = ventricular fibrillation   VT = ventricular tachycardia   WPW = Wolff-Parkinson-White

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