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J Wave Syndromes - Brugada and Early Repolarization Syndromes (Hardcover, 1st ed. 2016)
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J Wave Syndromes - Brugada and Early Repolarization Syndromes (Hardcover, 1st ed. 2016)
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This book delineates the state of the art of the diagnosis and
treatment of J wave syndromes, as well as where future research
needs to be directed. It covers basic science, translational and
clinical aspects of these syndromes. The authors are leading
experts in their respective fields, who have contributed
prominently to the literature concerning these topics. J wave
syndromes are one of the hottest topics in cardiology today.
Cardiac arrhythmias associated with Brugada syndrome (BrS) or an
early repolarization (ER) pattern in the inferior or infero-lateral
ECG leads are thought to be mechanistically linked to accentuation
of transient outward current (Ito)-mediated J waves. Although BrS
and ER syndrome (ERS) differ with respect to magnitude and lead
location of abnormal J waves, they are thought to represent a
continuous spectrum of phenotypic expression termed J wave
syndromes. ERS is divided into three subtypes with the most severe,
Type 3, displaying an ER pattern globally in the inferior, lateral
and right precordial leads. BrS has been linked to mutations in 19
different genes, whereas ERS has been associated with mutations in
7 different genes. There is a great deal of confusion as to how to
properly diagnose and treat the J wave syndromes as well as
confusion about the underlying mechanisms. The demonstration of
successful epicardial ablation of BrS has provided new therapeutic
options for the management of this syndrome for which treatment
alternatives are currently very limited, particularly in the case
of electrical storms caused by otherwise uncontrollable recurrent
VT/VF. An early repolarization pattern is observed in 2-5% of the
US population. While it is clear that the vast majority of
individuals exhibiting an ER pattern are not at risk for sudden
cardiac death, the challenge moving forward is to identify those
individuals who truly are at risk and to design safe and effective
treatments.
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