Familial sudden death syndrome with an abnormal signal-averaged electrocardiogram as a potential marker

Jeffrey W. Chambers*, Pablo Denes, Wendy Dahl, Douglas A. Olson, Daniel Galita, Michael J. Osborn, Jack L. Titus

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


Most familial sudden cardiac death syndromes are associated with structural heart disease or 12-lead electrocardiographic abnormalities. Additionally, the utility of signal-averaged electrocardiograms in patients with familial sudden death syndromes has not been examined. We studied a kindred with sudden death to determine whether they could be classified into any of the previously described syndromes and whether an abnormal signal-averaged electrocardiogram is a marker for this trait. Surviving family members had normal 12-lead electrocardiograms and echocardiograms. Two of the patients who died from ventricular arrhythmias had normal hearts on autopsy. Two surviving family members had a clinical history of arrhythmic events; both had abnormal signal-averaged electrocardiograms and inducible ventricular arrhythmias during electrophysiologic studies. The other family members had normal signal-averaged electrocardiograms. This familial sudden death syndrome appears to be unique because the patients have anatomically normal hearts and normal 12-lead electrocardiograms. An abnormal signal-averaged electrocardiogram may be a marker for the sudden death trait.

Original languageEnglish (US)
Pages (from-to)318-323
Number of pages6
JournalAmerican heart journal
Issue number2
StatePublished - Aug 1995

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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