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Title: The prevalence and significance of the Early Repolarization pattern in Sudden Arrhythmic Death Syndrome families
Authors: Mellor, Greg
Nelson, Christopher P.
Robb, Claire
Raju, Hariharan
Wijeyeratne, Yanushi
Hengstenberg, Christian
Hengstenberg, Wibke
Papadakis, Michael
Sharma, Sharma
Samani, Nilesh J.
Behr, Elijah R.
First Published: 2-Jun-2016
Publisher: American Heart Association
Citation: Circulation: Arrhythmia and Electrophysiology, 2016, 9 (6):e003960
Abstract: 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.
DOI Link: 10.1161/CIRCEP.116.003960
ISSN: 1941-3149
eISSN: 1941-3084
Version: Publisher Version
Status: Peer-reviewed
Type: Journal Article
Rights: Copyright © the authors, 2016. After an embargo period this will be an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License ( ), which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
Description: 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.
Appears in Collections:Published Articles, Dept. of Cardiovascular Sciences

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