Inheritance of Early Repolarization and Familial Malignant Forms

Clearly the presence of high-risk features with the ER pattern in these presumed ER syndrome relatives may portend a higher risk of death from other or more common cardiac causes other than of IVF (ER syndrome) per se. [...]the sudden death rate purely attributable to familial ER syndrome may be ove...

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Bibliographic Details
Published in:Journal of the American College of Cardiology 2013-01, Vol.61 (2), p.173-175
Main Authors: Krahn, Andrew D., MD, Obeyesekere, Manoj N., MBBS
Format: Article
Language:English
Subjects:
Arrhythmias, Cardiac - diagnosis
Arrhythmias, Cardiac - genetics
Cardiac arrhythmia
Cardiology
Cardiovascular
early repolarization
early repolarization syndrome
Electrocardiography
familial early repolarization
family screening
Female
Heart attacks
Humans
idiopathic ventricular fibrillation
Internal Medicine
Male
Mutation
Studies
sudden cardiac death
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Summary:Clearly the presence of high-risk features with the ER pattern in these presumed ER syndrome relatives may portend a higher risk of death from other or more common cardiac causes other than of IVF (ER syndrome) per se. [...]the sudden death rate purely attributable to familial ER syndrome may be overestimated. The key finding in this study is that ER associated with familial sudden death appears to be genetically mediated in an autosomal dominant inheritance pattern, with an ER phenotype that is indistinguishable from the much more common sporadic form associated with IVF and an electrocardiographic phenotype indistinguishable from the ER pattern reported in population studies that can modify arrhythmic risk or is benign.
ISSN:0735-1097
1558-3597
DOI:10.1016/j.jacc.2012.09.039