Atrial Arrhythmias in long-QT syndrome under daily life conditions: a nested case control study

ZELLERHOFF, STEPHAN; PISTULLI, RUDIN; MÖNNIG, GEROLD; HINTERSEER, MARTIN; BECKMANN, BRITT‐MARIA; KÖBE, JULIA; STEINBECK, GERHARD; KÄÄB, STEFAN; HAVERKAMP, WILHELM; FABRITZ, LARISSA; GRADAUS, RAINER; BREITHARDT, GÜNTER; SCHULZE‐BAHR, ERIC; BÖCKER, DIRK; KIRCHHOF, PAULUS

doi:10.1111/j.1540-8167.2008.01339.x

Cited by 71Cardiac electrophysiology and arrhythmiasCardiac Arrhythmias and TreatmentsCardiac pacing and defibrillation studies

Atrial Arrhythmias in long-QT syndrome under daily life conditions: a nested case control study

ZELLERHOFF, STEPHAN, PISTULLI, RUDIN, MÖNNIG, GEROLD, HINTERSEER, MARTIN, BECKMANN, BRITT‐MARIA, KÖBE, JULIA, STEINBECK, GERHARD, KÄÄB, STEFAN +7 more·2009·Journal of Cardiovascular Electrophysiology

DOI 10.1111/j.1540-8167.2008.01339.x

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Abstract

BACKGROUND: The long-QT syndromes (LQTS) are inherited electrical cardiomyopathies characterized by prolonged ventricular repolarization and ventricular arrhythmias. Several genetic reports have associated defects in LQTS-causing genes with atrial fibrillation (AF). We therefore studied whether atrial arrhythmias occur in patients with LQTS under daily-life conditions. METHODS: We systematically assessed atrial arrhythmias in LQTS patients and matched controls using implanted defibrillators or pacemakers as monitors of atrial rhythm in a nested case-control study. Twenty-one LQTS patients (3 male; 39 +/- 18 years old; 18 on beta blocker, ICD therapy duration 6.3 +/- 2.7 years; 4 LQT1, 6 LQT2, 2 LQT3) were matched to 21 control subjects (13 male; 50 +/- 19 years old; 3 on beta blocker; pacemaker therapy duration 8.5 +/- 5.5 years; 19 higher-degree AV block, 2 others). LQTS patients were identified by a systematic search of the LQTS patient databases in Münster and Munich. RESULTS: One-third (7 of 21) of the LQTS patients developed self-terminating atrial arrhythmias (atrial cycle lengths <250 ms). Only one control patient developed a single episode of postoperative AF (P < 0.05 vs LQTS). CONCLUSIONS: LQTS patients at high risk for ventricular arrhythmias may develop short-lasting atrial arrhythmias under daily-life conditions, suggesting that prolonged atrial repolarization may contribute to the initiation of AF.

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AF prevalence in long QT syndrome is 5-10%.
“Long QT syndrome KCNQ1 KCNH2 SCN5A ANK2 others IKs  IKr  INa  INa,K  Various effects 5-10% 846-850”
From 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS (2016) · cites this paper as [848]
Monogenic defects only account for 3-5% of all patients with AF, even in younger populations.
“Monogenic defects only account for 3-5% of all patients with AF, even in younger populations.846,848,[888][889][890]”
From 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS (2016) · cites this paper as [848]

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Authors: ZELLERHOFF, S., PISTULLI, R., MÖNNIG, G., HINTERSEER, M., BECKMANN, B., KÖBE, J., STEINBECK, G., KÄÄB, S., HAVERKAMP, W., FABRITZ, L., GRADAUS, R., BREITHARDT, G., SCHULZE‐BAHR, E., BÖCKER, D., & KIRCHHOF, P.
Affiliations: University of Münster (DE)
Ludwig-Maximilians-Universität München (DE)
Atrial Fibrillation NETwork (DE)
Charité – Universitätsmedizin Berlin (DE)
ORCID: PISTULLI, RUDIN
HINTERSEER, MARTIN
BECKMANN, BRITT‐MARIA
KÖBE, JULIA
STEINBECK, GERHARD
KÄÄB, STEFAN
HAVERKAMP, WILHELM
FABRITZ, LARISSA
BREITHARDT, GÜNTER
SCHULZE‐BAHR, ERIC
KIRCHHOF, PAULUS
Venue: Journal of Cardiovascular Electrophysiology
Publisher: Wiley
Published: 2009-03-23
Volume: 20
Issue: 4
Pages: 401-407
ISSN: 1045-3873 (ISSN-L)
1540-8167
DOI: 10.1111/j.1540-8167.2008.01339.x
OpenAlex: W2046450194
Cited by: 71
References: 33
Citations by year: 2026: 3 · 2025: 1 · 2024: 4 · 2023: 4 · 2021: 5 · 2020: 5 · 2019: 1 · 2018: 5 · 2017: 4 · 2016: 7 · 2015: 3 · 2014: 4 · 2013: 4 · 2012: 6
FWCI: 3.64
Type: journal article
Language: en
Open access: closed
Licence: onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations: Journal of Cardiovascular Electrophysiology (publishedVersion)
PubMed (publishedVersion)
University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
Topics: Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac pacing and defibrillation studies
Keywords: long QT syndrome, atrial arrhythmias, atrial fibrillation, atrial repolarization, LQT1, long QT syndrome type 2, long QT syndrome type 3, ventricular arrhythmia risk
MeSH terms: Activities of Daily Living, Adolescent, Adrenergic beta-Antagonists, Adult, Aged, Aged, 80 and over, Atrial Fibrillation, Child, Electrocardiography, Female, Germany, Humans, Long QT Syndrome, Male, Middle Aged, Pacemaker, Artificial, Time Factors, Case-Control Studies, Treatment Outcome, Defibrillators, Implantable, Young Adult

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Atrial Arrhythmias in long-QT syndrome under daily life conditions: a nested case control study

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