Prolonged atrial action potential durations and polymorphic atrial tachyarrhythmias in patients with long QT syndrome
Abstract
UNLABELLED: Atrial APD and Polymorphic AT in LQTS. INTRODUCTION: Prolongation of the QT interval and torsades de pointes tachycardias due to altered expression or function of repolarizing ion channels are the hallmark of congenital long QT syndrome (LQTS). The same ion channels also contribute to atrial repolarization, and familial atrial fibrillation may be associated with a mutated KVLQT1 gene. We therefore assessed atrial action potential characteristics and atrial arrhythmias in LQTS patients. METHODS AND RESULTS: Monophasic action potentials (MAPs) were simultaneously recorded from the right atrial appendage and the inferolateral right atrium in 10 patients with LQTS (8 with identifiable genotype) and compared to 7 control patients. Atrial arrhythmias also were compared to MAPs recorded in patients with persistent (n = 10) and induced (n = 4) atrial fibrillation. Atrial action potential durations (APD) and effective refractory periods (ERP) were prolonged in LQTS patients at cycle lengths of 300 to 500 msec (APD prolongation 30-41 msec; ERP prolongation 26-52 msec; all P < 0.05). Short episodes of polymorphic atrial tachyarrhythmias (polyAT, duration 4-175 sec) occurred spontaneously or during pauses after pacing in 5 of 10 LQTS patients, but not in controls (P < 0.05). P waves showed undulating axis during polyAT. Cycle lengths of polyAT were longer than during persistent and induced atrial fibrillation. Afterdepolarizations preceded polyAT in 2 patients. The electrical restitution curve was shifted to longer APD in LQTS patients and to even longer APD in LQTS patients with polyAT. CONCLUSION: This group of LQTS patients has altered atrial electrophysiology: action potentials are prolonged, and polyAT occurs. PolyAT appears to be a specific arrhythmia of LQTS reminiscent of an atrial form of "torsades de pointes".
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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 [847]
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- Authors
- KIRCHHOF, P., ECKARDT, L., FRANZ, M. R., MÖNNIG, G., LOH, P., WEDEKIND, H., SCHULZE‐BAHR, E., BREITHARDT, G., & HAVERKAMP, W.
- Affiliations
- University of Münster (DE)
- Georgetown University (US)
- Corresponding author
- KIRCHHOF, PAULUS
- ORCID
- Venue
- Journal of Cardiovascular Electrophysiology
- Publisher
- Wiley
- Published
- 2003-09-19
- Volume
- 14
- Issue
- 10
- Pages
- 1027-1033
- ISSN
- 1045-3873 (ISSN-L)
- 1540-8167
- OpenAlex
- W2089858939
- Cited by
- 124
- References
- 34
- Citations by year
- 2026: 3 · 2025: 1 · 2024: 6 · 2023: 4 · 2022: 2 · 2021: 6 · 2020: 7 · 2019: 4 · 2018: 5 · 2017: 6 · 2016: 14 · 2015: 1 · 2014: 1 · 2013: 6 · 2012: 4
- FWCI
- 3.73
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Cardiovascular Electrophysiology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
- Keywords
- long QT syndrome, atrial action potential duration, atrial fibrillation, atrial repolarization, effective refractory period, KCNQ1, afterdepolarizations, electrical restitution, monophasic action potential, atrial electrophysiology, torsades de pointes
- MeSH terms
- Action Potentials, Adolescent, Adult, Atrial Fibrillation, Electrocardiography, Female, Heart Atria, Heart Conduction System, Humans, Long QT Syndrome, Male, Middle Aged, Polymorphism, Genetic, Tachycardia, Ectopic Atrial
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