Open AccessElectrophysiologic Substrate in Congenital Long QT Syndrome
Author(s) -
Ramya Vijayakumar,
Jennifer N. Avari Silva,
Kavit A. Desouza,
Robert L. Abraham,
Maria Ström,
Frédéric Sacher,
George F. Van Hare,
Michel Haı̈ssaguerre,
Dan M. Roden,
Yoram Rudy
Publication year - 2014
Publication title -
circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.795
H-Index - 607
eISSN - 1524-4539
pISSN - 0009-7322
DOI - 10.1161/circulationaha.114.011359
Subject(s) - repolarization , medicine , cardiology , long qt syndrome , qt interval , asymptomatic , electrophysiology , electrocardiography , sudden cardiac death , sudden death , anesthesia
Congenital Long QT syndrome (LQTS) is an arrhythmogenic disorder that causes syncope and sudden death. Although its genetic basis has become well-understood, the mechanisms whereby mutations translate to arrhythmia susceptibility in the in situ human heart have not been fully defined. We used noninvasive ECG imaging to map the cardiac electrophysiological substrate and examine whether LQTS patients display regional heterogeneities in repolarization, a substrate that promotes arrhythmogenesis.
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