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The prevalence and significance of the Early Repolarization pattern in Sudden Arrhythmic Death Syndrome families

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journal contribution
posted on 12.05.2016, 14:22 by Greg Mellor, Christopher P. Nelson, Claire Robb, Hariharan Raju, Yanushi Wijeyeratne, Christian Hengstenberg, Wibke Hengstenberg, Michael Papadakis, Sharma Sharma, Nilesh J. Samani, Elijah R. Behr
Background: The early repolarization (ER) pattern is associated with sudden death and has been shown to be heritable. Its significance when identified in families affected by sudden arrhythmic death syndrome (SADS) remains unclear. Methods and Results: We analyzed 12-lead ECGs of 401 first-degree relatives of individuals who had died from SADS. The prevalence of ER patterns was compared to family-clustered controls. ER was more common in SADS family members than in controls (21% vs. 8%, OR 5.14, 95% CI 3.37-7.84) independent of the presence of a familial cardiac diagnosis. Both ascending and horizontal ER patterns were more common. In addition ER was investigated for associations with findings from ajmaline provocation (n=332), exercise ECG (n=304) and signal-averaged ECG (n=118) when performed. ER was associated with a trend toward late depolarization, in general was suppressed with exercise and was unaffected by ajmaline. Inferior and horizontal patterns were, however, more likely to persist during exercise. Augmentation of ER with ajmaline was rare. Conclusions: The ER pattern is more common in SADS family members than controls adjusted in particular for relatedness. The increased prevalence is irrespective of ER subtype and the presence of other inherited arrhythmia syndromes. ER may therefore represent an underlying heritable arrhythmia syndrome or risk factor for sudden death in the context of other cardiac pathology. The differing response of ER subtypes to exercise and ajmaline provocation suggests underlying mechanisms of both abnormal repolarization and depolarization.

History

Citation

Circulation: Arrhythmia and Electrophysiology, 2016, 9 (6):e003960

Author affiliation

/Organisation/COLLEGE OF MEDICINE, BIOLOGICAL SCIENCES AND PSYCHOLOGY/School of Medicine/Department of Cardiovascular Sciences

Version

VoR (Version of Record)

Published in

Circulation: Arrhythmia and Electrophysiology

Publisher

American Heart Association

issn

1941-3149

eissn

1941-3084

Acceptance date

03/05/2016

Copyright date

2016

Available date

02/12/2016

Publisher version

http://circep.ahajournals.org/content/9/6/e003960

Notes

The file associated with this record is embargoed until 6 months after the date of publication. The final published version may be available through the links above.

Language

en