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Quantitative T-wave morphology assessment from surface ECG is linked with cardiac events risk in genotype-positive KCNH2 mutation carriers with normal QTc values

Cortez, Daniel LU ; Zareba, Wojciech ; McNitt, Scott ; Polonsky, Bronislava ; Rosero, Spencer Z. and Platonov, Pyotr G. LU (2019) In Journal of Cardiovascular Electrophysiology 30(12). p.2907-2913
Abstract

Introduction: Long QT syndrome (LQTS) mutation carriers have elevated the risk of cardiac events even in the absence of QTc prolongation; however, mutation penetrance in patients with normal QTc may be reflected in abnormal T-wave shape, particularly in KCNH2 mutation carriers. We aimed to assess whether the magnitude of a three-dimensional T-wave vector (TwVM) will identify KCNH2-mutation carriers with normal QTc at risk for cardiac events. Methods: Adult LQT2 patients with QTc < 460 ms in men and <470 ms in women (n = 113, age 42 ± 16 years, 43% male) were compared with genotype-negative family members (n = 1007). The TwVM was calculated using T-wave amplitudes in leads V6, II, and V2 as the square root of (TV62 +... (More)

Introduction: Long QT syndrome (LQTS) mutation carriers have elevated the risk of cardiac events even in the absence of QTc prolongation; however, mutation penetrance in patients with normal QTc may be reflected in abnormal T-wave shape, particularly in KCNH2 mutation carriers. We aimed to assess whether the magnitude of a three-dimensional T-wave vector (TwVM) will identify KCNH2-mutation carriers with normal QTc at risk for cardiac events. Methods: Adult LQT2 patients with QTc < 460 ms in men and <470 ms in women (n = 113, age 42 ± 16 years, 43% male) were compared with genotype-negative family members (n = 1007). The TwVM was calculated using T-wave amplitudes in leads V6, II, and V2 as the square root of (TV62 + TII2 + (0.5*TV2)2). Cox regression analysis adjusted for gender and time-dependent beta-blocker use was performed to assess cardiac event (CE) risk, defined as syncope, aborted cardiac arrest, implantable cardioverter-defibrillator therapy, or sudden death. Results: Dichotomized by median of 0.30 mV, lower TwVM was associated with elevated CE risk compared to those with high TwVM (HR = 2.95, 95% CI, 1.25-6.98, P =.014) and also remained significant after including sex and time-dependent beta-blocker usage in the Cox regression analysis (HR = 2.64, 95% CI, 1.64-4.24, P <.001). However, these associations were found only in women but not in men who had low event rates. Conclusion: T-wave morphology quantified as repolarization vector magnitude using T-wave amplitudes retrieved from standard 12-lead electrocardiogram predicts cardiac events risk in LQT2 women and appears useful for risk stratification of KCNH2-mutation carriers without QTc prolongation.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
cardiac events, long QT syndrome, T-wave vector magnitude
in
Journal of Cardiovascular Electrophysiology
volume
30
issue
12
pages
2907 - 2913
publisher
Wiley-Blackwell
external identifiers
  • pmid:31579959
  • scopus:85074089075
ISSN
1045-3873
DOI
10.1111/jce.14210
language
English
LU publication?
yes
id
8b7d4ce0-a4bc-443b-a22f-fa3ef7dd86ee
date added to LUP
2019-11-07 12:33:19
date last changed
2020-07-01 05:24:14
@article{8b7d4ce0-a4bc-443b-a22f-fa3ef7dd86ee,
  abstract     = {<p>Introduction: Long QT syndrome (LQTS) mutation carriers have elevated the risk of cardiac events even in the absence of QTc prolongation; however, mutation penetrance in patients with normal QTc may be reflected in abnormal T-wave shape, particularly in KCNH2 mutation carriers. We aimed to assess whether the magnitude of a three-dimensional T-wave vector (TwVM) will identify KCNH2-mutation carriers with normal QTc at risk for cardiac events. Methods: Adult LQT2 patients with QTc &lt; 460 ms in men and &lt;470 ms in women (n = 113, age 42 ± 16 years, 43% male) were compared with genotype-negative family members (n = 1007). The TwVM was calculated using T-wave amplitudes in leads V6, II, and V2 as the square root of (TV6<sup>2</sup> + TII<sup>2</sup> + (0.5*TV2)<sup>2</sup>). Cox regression analysis adjusted for gender and time-dependent beta-blocker use was performed to assess cardiac event (CE) risk, defined as syncope, aborted cardiac arrest, implantable cardioverter-defibrillator therapy, or sudden death. Results: Dichotomized by median of 0.30 mV, lower TwVM was associated with elevated CE risk compared to those with high TwVM (HR = 2.95, 95% CI, 1.25-6.98, P =.014) and also remained significant after including sex and time-dependent beta-blocker usage in the Cox regression analysis (HR = 2.64, 95% CI, 1.64-4.24, P &lt;.001). However, these associations were found only in women but not in men who had low event rates. Conclusion: T-wave morphology quantified as repolarization vector magnitude using T-wave amplitudes retrieved from standard 12-lead electrocardiogram predicts cardiac events risk in LQT2 women and appears useful for risk stratification of KCNH2-mutation carriers without QTc prolongation.</p>},
  author       = {Cortez, Daniel and Zareba, Wojciech and McNitt, Scott and Polonsky, Bronislava and Rosero, Spencer Z. and Platonov, Pyotr G.},
  issn         = {1045-3873},
  language     = {eng},
  month        = {10},
  number       = {12},
  pages        = {2907--2913},
  publisher    = {Wiley-Blackwell},
  series       = {Journal of Cardiovascular Electrophysiology},
  title        = {Quantitative T-wave morphology assessment from surface ECG is linked with cardiac events risk in genotype-positive KCNH2 mutation carriers with normal QTc values},
  url          = {http://dx.doi.org/10.1111/jce.14210},
  doi          = {10.1111/jce.14210},
  volume       = {30},
  year         = {2019},
}