Prevalence of early-onset atrial fibrillation in congenital long QT syndrome
Open accessAbstract
Background The prevalence of atrial fibrillation (AF) in the young (age <50 years) is 0.1%, or 1:1,000 persons. Mutations in KCNQ1-, KCNH2-, and KCNA5-encoded potassium channels and SCN5A-encoded sodium channels have been reported in familial AF. A mechanism of atrial torsade has been suggested to occur in patients with congenital long QT syndrome (LQTS).Objective The purpose of this study was to determine the prevalence of AF in patients with congenital LQTS.Methods History of documented AF was sought from two independent cohorts. One cohort consisted of 252 consecutive patients (146 females and 106 males, average age at diagnosis 23 +/- 16 years, QTc 465 +/- 51 ms) with genetically proven LQTS seen at Mayo's LQTS Clinic. The second cohort consisted of 205 consecutive patients (133 females and 72 males, average age at testing 23 +/- 16 years, QTc 479 +/- 51 ms) with a positive FAMILION genetic test (PGxHealth) for LQTS.Results Early-onset AF was documented in 8 (1.7%) of 457 patients, including 6 (2.4%) of 252 patients seen at Mayo and 2 (1%) of 205 patients with a positive FAMILION test. Five (2.4%) of 211 patients with LQT1-susceptibility mutations had documented AF, compared to 0 of 174 patients with LQT2, 1 of 59 patients with LQT3, 1 of 1 patient with Andersen-Tawil syndrome, and 1 of 34 patients with multiple mutations. The average age at diagnosis of AF of the six patients evaluated at Mayo was 24.3 years (range 4-46 years). Early-onset AF (age <50 years) was significantly more common in patients with LQTS compared to population-based prevalence statistics (P <.001, relative risk 17.5).Conclusion Compared to the background prevalence of 0.1%, early-onset AF was observed in almost 2% of patients with genetically proven LQTS and should be viewed as an uncommon but possible LQT-related dysrhythmia. Clinical complaints of palpitations warrant thorough assessment in patients with LQTS.
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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 [846]
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 [846]
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- Authors
- Johnson, J. N., Tester, D. J., Perry, J., Salisbury, B. A., Reed, C. R., & Ackerman, M. J.
- Affiliations
- Mayo Clinic (US)
- Corresponding author
- Ackerman, Michael J.
- ORCID
- Venue
- Heart Rhythm
- Publisher
- Elsevier BV
- Published
- 2008-05
- Volume
- 5
- Issue
- 5
- Pages
- 704-709
- ISSN
- 1547-5271 (ISSN-L)
- PMC
- PMC3940082
- OpenAlex
- W2063459798
- Cited by
- 179
- References
- 22
- Citations by year
- 2026: 7 · 2025: 2 · 2024: 15 · 2023: 10 · 2022: 2 · 2021: 9 · 2020: 14 · 2019: 7 · 2018: 7 · 2017: 10 · 2016: 15 · 2015: 14 · 2014: 8 · 2013: 18 · 2012: 11
- FWCI
- 7.06
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Heart Rhythm (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- CJ Foundation for SIDS
- National Institutes of Health (HD42569)
- American Heart Association
- Dr. Scholl Foundation
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Keywords
- early-onset atrial fibrillation, congenital long QT syndrome, atrial fibrillation prevalence, LQT1, long QT syndrome type 2, long QT syndrome type 3, Andersen-Tawil syndrome, potassium channel mutations, SCN5A, young adults, palpitations, familial atrial fibrillation, QTc prolongation
- MeSH terms
- Adolescent, Adult, Atrial Fibrillation, Child, Female, Humans, Long QT Syndrome, Male, Sodium Channels, Prevalence, Age of Onset, Genetic Predisposition to Disease, Potassium Channels, Voltage-Gated
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