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(Circulation. 2005;112:2220-2221.)
© 2005 American Heart Association, Inc.

Editorial

Sudden Cardiac Arrest From Primary Electrical Diseases

Provoking Concealed Arrhythmogenic Syndromes

N.A. Mark Estes, III, MD

From the Tufts University School of Medicine, Boston, Mass.

Correspondence to N.A. Mark Estes, III, MD, Director, Cardiac Arrhythmia Service, New England Medical Center Hospital, 750 Washington St, Boston, MA 02493. E-mail Nestes@tufts-nemc.org

Key Words: Editorials • arrhythmia • cardiac arrest • diagnosis • death, sudden

An extract of the first 250 words of the full text is provided, because this article has no abstract.

Cardiac arrest most commonly occurs as a consequence of acute myocardial ischemia or develops with identifiable substrate such as scar because of previous myocardial infarction, cardiomyopathy, or hypertrophy.^1,2 Despite extensive noninvasive and invasive evaluation, cardiac arrest remains unexplained without evidence of structural heart disease in 10% of individuals surviving sudden cardiac arrest.^1,2 Autopsy data confirm that a similar minority of victims of sudden death have no identifiable cardiac abnormality at postmortem evaluation.^1,2 It is now recognized that inherited electrophysiological abnormalities, termed primary electrical diseases, are the common underlying cause of these unexplained cardiac arrests. Such tragic events disproportionately affect people <40 years old.^1,2 These conditions include Wolff-Parkinson White syndrome, long QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT).^3–9 The remaining patients without such distinct electrophysiological abnormalities are generally categorized as having idiopathic ventricular fibrillation.^1–3 It is evident that implementing appropriate screening and therapeutic strategies is dependent on accurate identification of those affected. The diagnosis of these conditions remains a vexing challenge for clinicians because the conditions commonly are not identifiable with standard clinical evaluation. In this respect, they are considered concealed arrhythmogenic syndromes, which manifest only intermittently or with specific provocative maneuvers.

Article p 2228

Although Brugada syndrome can present with the characteristic right bundle-branch block and persistent ST-segment elevation, latent or intermittent forms of the condition commonly make the diagnosis difficult.^4,5 The ECG can be modulated by changes in autonomic balance, body temperature, glucose levels, and the administration of drugs.^4,5 The subtle ST-segment shifts can be difficult . . . [Full Text of this Article]