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Atrial Fibrillation in Long QT Syndrome by Genotype

Platonov, Pyotr G. LU ; McNitt, Scott ; Polonsky, Bronislava ; Rosero, Spencer Z. and Zareba, Wojciech (2019) In Circulation: Arrhythmia and Electrophysiology 12(10).
Abstract

BACKGROUND: Long QT syndrome (LQTS) is caused by the abnormal function of ion channels, which may also affect atrial electrophysiology and be associated with the risk of atrial fibrillation (AF). However, large-scale studies of AF risk among patients with LQTS and its relation to LQTS manifestations are lacking. We aimed to assess the risk of AF and its relationship to the LQTS genotype and the long-term prognosis in patients with LQTS. METHODS: Genotype-positive patients with LQTS (784 LQT1, 746 LQT2, and 233 LQT3) were compared with 2043 genotype-negative family members. Information on the occurrence of AF was based on physician-reported ECG-verified events. Multivariate Cox proportional hazards regression analyses were performed for... (More)

BACKGROUND: Long QT syndrome (LQTS) is caused by the abnormal function of ion channels, which may also affect atrial electrophysiology and be associated with the risk of atrial fibrillation (AF). However, large-scale studies of AF risk among patients with LQTS and its relation to LQTS manifestations are lacking. We aimed to assess the risk of AF and its relationship to the LQTS genotype and the long-term prognosis in patients with LQTS. METHODS: Genotype-positive patients with LQTS (784 LQT1, 746 LQT2, and 233 LQT3) were compared with 2043 genotype-negative family members. Information on the occurrence of AF was based on physician-reported ECG-verified events. Multivariate Cox proportional hazards regression analyses were performed for ages 0 to 60 and after 60 years (reflecting an early and late-onset of AF) to assess the risk of incident AF by genotype and the relationship of AF to the risk of cardiac events defined as syncope, documented torsades de pointes, and aborted cardiac arrest or sudden cardiac death. RESULTS: In patients followed from birth to 60 years of age, patients with LQT3 had an increased risk of AF compared with genotype-negative family members (hazard ratio=6.62; 95% CI, 2.04-21.49; P<0.001), while neither LQT1 nor LQT2 demonstrated increased AF risk. After the age of 60 years, patients with LQT2 had significantly lower risk of AF compared with genotype-negative controls (hazard ratio=0.07; 95% CI, 0.01-0.53, P=0.011). AF was a significant predictor of cardiac events in patients with LQT3 through the age of 60 (hazard ratio=5.38; 95% CI, 1.17-24.82; P=0.031). CONCLUSIONS: Our data demonstrate an increased risk of early age AF in patients with LQT3 and also indicate a protective effect of the LQT2 genotype in it's association with a decreased risk of AF after the age of 60.

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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
atrial fibrillation, electrophysiology, genotype, long QT syndrome, sudden cardiac death
in
Circulation: Arrhythmia and Electrophysiology
volume
12
issue
10
article number
e007213
publisher
Lippincott Williams & Wilkins
external identifiers
  • scopus:85073176512
  • pmid:31610692
ISSN
1941-3084
DOI
10.1161/CIRCEP.119.007213
language
English
LU publication?
yes
id
bfbf2abb-fa36-455f-970c-52343de3a5ce
date added to LUP
2019-10-22 14:12:58
date last changed
2024-03-04 04:27:03
@article{bfbf2abb-fa36-455f-970c-52343de3a5ce,
  abstract     = {{<p>BACKGROUND: Long QT syndrome (LQTS) is caused by the abnormal function of ion channels, which may also affect atrial electrophysiology and be associated with the risk of atrial fibrillation (AF). However, large-scale studies of AF risk among patients with LQTS and its relation to LQTS manifestations are lacking. We aimed to assess the risk of AF and its relationship to the LQTS genotype and the long-term prognosis in patients with LQTS. METHODS: Genotype-positive patients with LQTS (784 LQT1, 746 LQT2, and 233 LQT3) were compared with 2043 genotype-negative family members. Information on the occurrence of AF was based on physician-reported ECG-verified events. Multivariate Cox proportional hazards regression analyses were performed for ages 0 to 60 and after 60 years (reflecting an early and late-onset of AF) to assess the risk of incident AF by genotype and the relationship of AF to the risk of cardiac events defined as syncope, documented torsades de pointes, and aborted cardiac arrest or sudden cardiac death. RESULTS: In patients followed from birth to 60 years of age, patients with LQT3 had an increased risk of AF compared with genotype-negative family members (hazard ratio=6.62; 95% CI, 2.04-21.49; P&lt;0.001), while neither LQT1 nor LQT2 demonstrated increased AF risk. After the age of 60 years, patients with LQT2 had significantly lower risk of AF compared with genotype-negative controls (hazard ratio=0.07; 95% CI, 0.01-0.53, P=0.011). AF was a significant predictor of cardiac events in patients with LQT3 through the age of 60 (hazard ratio=5.38; 95% CI, 1.17-24.82; P=0.031). CONCLUSIONS: Our data demonstrate an increased risk of early age AF in patients with LQT3 and also indicate a protective effect of the LQT2 genotype in it's association with a decreased risk of AF after the age of 60.</p>}},
  author       = {{Platonov, Pyotr G. and McNitt, Scott and Polonsky, Bronislava and Rosero, Spencer Z. and Zareba, Wojciech}},
  issn         = {{1941-3084}},
  keywords     = {{atrial fibrillation; electrophysiology; genotype; long QT syndrome; sudden cardiac death}},
  language     = {{eng}},
  number       = {{10}},
  publisher    = {{Lippincott Williams & Wilkins}},
  series       = {{Circulation: Arrhythmia and Electrophysiology}},
  title        = {{Atrial Fibrillation in Long QT Syndrome by Genotype}},
  url          = {{http://dx.doi.org/10.1161/CIRCEP.119.007213}},
  doi          = {{10.1161/CIRCEP.119.007213}},
  volume       = {{12}},
  year         = {{2019}},
}