Familial sudden death syndrome with an abnormal signal-averaged electrocardiogram as a potential marker.
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.
Chambers
J W
JW
Cardiology Section, St. Paul Ramsey Medical Center, Minn, USA.
Denes
P
P
Dahl
W
W
Olson
D A
DA
Galita
D
D
Osborn
M J
MJ
Titus
J L
JL
D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000235:genetics; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004562:Electrocardiography; D005192:Family Health; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012815:Signal Processing, Computer-Assisted; D013577:Syndrome