Critical Care: New cardiac arrhythmia syndrome is identified

August 2004 
Volume 34  Number 8
Pages 32cc8 - 32cc8
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    An inherited cardiac arrhythmia syndrome that can lead to sudden death in young people has been identified by a team of researchers from Duke University Medical Center in Durham, N.C., and the Howard Hughes Medical Institute in Chevy Chase, Md.

    The syndrome is related to mutations in the ankyrin-B gene, which encodes a protein involved in regulating the heart's electrical activity. The arrhythmias previously had been grouped with long QT syndrome arrhythmias, but researchers said some patients with the ankyrin-B mutation didn't have prolonged QT intervals. Because ankyrin-B is found in cardiac nerve cells as well as muscle cells of the atria and ventricles, researchers think it may play an important role in other areas of cardiac function.

    They recommend genetic testing for all family members of patients who die of sudden cardiac death, and they add that beta-blockers appear to be effective in controlling these arrhythmias. ...

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