{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T03:10:39Z","timestamp":1782270639061,"version":"3.54.5"},"reference-count":34,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,7,31]],"date-time":"2024-07-31T00:00:00Z","timestamp":1722384000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cardiovasc electrophysiol"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Early\u2010onset atrial fibrillation (AF) has already been observed in approximately 2% of patients with genetically proven long QT syndrome (LQTS). This frequency is higher than population\u2010based estimates of early\u2010onset AF. However, the concomitant expression of AF in LQTS is likely underestimated. The purpose of this study was to examine the clinical presentation, genetic background, and outcomes of a cohort of patients with LQTS and early\u2010onset AF referred to a single tertiary center.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Twenty\u2010seven patients diagnosed with congenital LQTS were included in the study based on the documentation of early\u2010onset (age \u226450 years) clinical or subclinical AF episodes in all available medical records, including standard electrocardiograms, wearable monitor or cardiac implantable electronic devices.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seventeen patients experienced clinical AF during the follow\u2010up period. Subclinical AF was detected in 10 patients through insertable or wearable cardiac monitors. In our series, the mean heart rate during AF episodes was found to be relatively low despite the patients' young age and the low or minimal effective doses of beta\u2010blockers used for QTc interval control. All patients exhibiting LQTS and early\u2010onset AF were genotype positive, carrying mutations in the KCNQ1 (66%), KCNH2, KCNE1, and SCN5A genes. Notably, most of these patients carried the same p.(R231C) mutation in the KCNQ1 gene (59%) and were from the same families, suggesting concurrent expression of familial AF and LQTS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>LQTS patients are prone to developing clinical and subclinical AF, even at a younger age. The occurrence of early\u2010onset AF in the LQTS population could be more frequent than previously assumed. AF should be considered as a potential dysrhythmia related to LQTS. Our study emphasizes the importance of carefully researching clinical and\/or subclinical episodes of AF through strict heart rhythm monitoring in the LQTS population.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jce.16384","type":"journal-article","created":{"date-parts":[[2024,7,31]],"date-time":"2024-07-31T11:06:10Z","timestamp":1722423970000},"page":"1941-1951","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical presentation and genetic characterization of early\u2010onset atrial fibrillation in patients affected by long QT syndrome: A single\u2010center experience"],"prefix":"10.1111","volume":"35","author":[{"given":"Berardo","family":"Sarubbi","sequence":"first","affiliation":[{"name":"Adult Congenital Heart Disease and Congenital and Familial Arrhythmias Unit Monaldi Hospital Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni Domenico","family":"Ciriello","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease and Congenital and Familial Arrhythmias Unit Monaldi Hospital Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferdinando","family":"Barretta","sequence":"additional","affiliation":[{"name":"Dipartimento di Biochimica e Biotecnologie Mediche University of Naples \u201cFederico II\u201d Naples Italy"},{"name":"CEINGE\u2010Biotecnologie Avanzate s.c.ar.l. 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