Pyotr G Platonov1,2, Scott McNitt2, Bronislava Polonsky2, Spencer Z Rosero2, Wojciech Zareba2. 1. Department of Cardiology, Clinical Sciences, Lund University, Sweden (P.G.P.). 2. Clinical Cardiovascular Research Center, Division of Cardiology, University of Rochester Medical Center, NY (P.G.P., S.M., B.P., S.Z.R., W.Z.).
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 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.
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 ageAF 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.
Entities:
Keywords:
atrial fibrillation; electrophysiology; genotype; long QT syndrome; sudden cardiac death
Authors: Norbert Jost; László Virág; Miklós Bitay; János Takács; Csaba Lengyel; Péter Biliczki; Zsolt Nagy; Gábor Bogáts; David A Lathrop; Julius G Papp; András Varró Journal: Circulation Date: 2005-08-29 Impact factor: 29.690
Authors: Jonathan N Johnson; David J Tester; James Perry; Benjamin A Salisbury; Carol R Reed; Michael J Ackerman Journal: Heart Rhythm Date: 2008-02-08 Impact factor: 6.343
Authors: Jonas Bille Nielsen; Claus Graff; Adrian Pietersen; Bent Lind; Johannes Jan Struijk; Morten Salling Olesen; Stig Haunsø; Thomas Alexander Gerds; Jesper Hastrup Svendsen; Lars Køber; Anders Gaarsdal Holst Journal: J Am Coll Cardiol Date: 2013-04-12 Impact factor: 24.094
Authors: Hanumantha R Jogu; Wesley T O'Neal; Stephen T Broughton; Amit J Shah; Zhu-Ming Zhang; Elsayed Z Soliman Journal: Ann Noninvasive Electrocardiol Date: 2016-08-12 Impact factor: 1.468
Authors: Rocío Fernández-Serra; Emma Martínez-Alonso; Alberto Alcázar; Mourad Chioua; José Marco-Contelles; Ricardo Martínez-Murillo; Milagros Ramos; Gustavo V Guinea; Daniel González-Nieto Journal: Int J Mol Sci Date: 2022-07-04 Impact factor: 6.208
Authors: África García-Roy; Ana Sarsa-Gómez; Fátima Méndez-López; Blanca Urdin-Muñoz; María Antonia Sánchez-Calavera Journal: Int J Environ Res Public Health Date: 2022-05-31 Impact factor: 4.614
Authors: Aleksei V Mikhailov; Anuradha Kalyanasundaram; Ning Li; Shane S Scott; Esthela J Artiga; Megan M Subr; Jichao Zhao; Brian J Hansen; John D Hummel; Vadim V Fedorov Journal: J Mol Cell Cardiol Date: 2020-10-29 Impact factor: 5.000
Authors: Jan-Thorben Sieweke; Jan Hagemus; Saskia Biber; Dominik Berliner; Gerrit M Grosse; Sven Schallhorn; Tobias Jonathan Pfeffer; Anselm A Derda; Jonas Neuser; Johann Bauersachs; Udo Bavendiek Journal: Front Cardiovasc Med Date: 2022-03-08
Authors: Maurice Pradella; Constantin Anastasopoulos; Shan Yang; Manuela Moor; Patrick Badertscher; Julian E Gehweiler; Florian Spies; Philip Haaf; Michael Zellweger; Gregor Sommer; Bram Stieltjes; Jens Bremerich; Stefan Osswald; Michael Kühne; Christian Sticherling; Sven Knecht Journal: PLoS One Date: 2022-08-15 Impact factor: 3.752
Authors: Zhishan Sun; Chengming Fan; Long Song; Hao Zhang; Zenan Jiang; Haoyu Tan; Yaqin Sun; Liming Liu Journal: Front Cardiovasc Med Date: 2022-07-15
Authors: Felix K Wegner; Robert M Radke; Christian Ellermann; Julian Wolfes; Kevin Willy; Philipp S Lange; Gerrit Frommeyer; Helmut Baumgartner; Lars Eckardt; Gerhard-Paul Diller; Stefan Orwat Journal: J Pers Med Date: 2022-03-14