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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Front+Physiol
2016 ; 7
(ä): 467
Nephropedia Template TP
gab.com Text
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English Wikipedia
Electrophysiological Mechanisms of Brugada Syndrome: Insights from Pre-clinical
and Clinical Studies
#MMPMID27803673
Tse G
; Liu T
; Li KH
; Laxton V
; Chan YW
; Keung W
; Li RA
; Yan BP
Front Physiol
2016[]; 7
(ä): 467
PMID27803673
show ga
Brugada syndrome (BrS), is a primary electrical disorder predisposing affected
individuals to sudden cardiac death via the development of ventricular
tachycardia and fibrillation (VT/VF). Originally, BrS was linked to mutations in
the SCN5A, which encodes for the cardiac Na(+) channel. To date, variants in 19
genes have been implicated in this condition, with 11, 5, 3, and 1 genes
affecting the Na(+), K(+), Ca(2+), and funny currents, respectively. Diagnosis of
BrS is based on ECG criteria of coved- or saddle-shaped ST segment elevation
and/or T-wave inversion with or without drug challenge. Three hypotheses based on
abnormal depolarization, abnormal repolarization, and current-load-mismatch have
been put forward to explain the electrophysiological mechanisms responsible for
BrS. Evidence from computational modeling, pre-clinical, and clinical studies
illustrates that molecular abnormalities found in BrS lead to alterations in
excitation wavelength (?), which ultimately elevates arrhythmic risk. A major
challenge for clinicians in managing this condition is the difficulty in
predicting the subset of patients who will suffer from life-threatening VT/VF.
Several repolarization risk markers have been used thus far, but these neglect
the contributions of conduction abnormalities in the form of slowing and
dispersion. Indices incorporating both repolarization and conduction and based on
the concept of ? have recently been proposed. These may have better predictive
values than the existing markers.