{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T18:27:48Z","timestamp":1788546468812,"version":"build-2803163510"},"reference-count":46,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,3,24]],"date-time":"2020-03-24T00:00:00Z","timestamp":1585008000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Dutch Epilepsy Foundation"},{"name":"Christelijke Vereniging voor de Verpleging van Lijders aan Epilepsie"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2020,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>We ascertained the prevalence of ictal arrhythmias to explain the high rate of sudden unexpected death in epilepsy (SUDEP) in Dravet syndrome (DS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We selected cases with clinical DS, \u22656\u00a0years, <jats:italic>SCN1A<\/jats:italic> mutation, and\u00a0\u22651 seizure\/week. Home\u2010based ECG recordings were performed for 20\u00a0days continuously. Cases were matched for age and sex to two epilepsy controls with no DS and\u00a0\u22651 major motor seizure during video\u2010EEG. We determined the prevalence of peri\u2010ictal asystole, bradycardia, QTc changes, and effects of convulsive seizures (CS) on heart rate, heart rate variability (HRV), and PR\/QRS. Generalized estimating equations were used to account for multiple seizures within subjects, seizure type, and sleep\/wakefulness.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We included 59 cases. Ictal recordings were obtained in 45 cases and compared to 90 controls. We analyzed 547 seizures in DS (300 CS) and 169 in controls (120 CS). No asystole occurred. Postictal bradycardia was more common in controls (<jats:italic>n<\/jats:italic>\u00a0=\u00a011, 6.5%) than cases (<jats:italic>n<\/jats:italic>\u00a0=\u00a04, 0.7%; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.002). Peri\u2010ictal QTc\u2010lengthening (\u226560ms) occurred more frequently in DS (<jats:italic>n<\/jats:italic>\u00a0=\u00a064, 12%) than controls (<jats:italic>n<\/jats:italic>\u00a0=\u00a08, 4.7%, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.048); pathologically prolonged QTc was rare (once in each group). In DS, interictal HRV was lower compared to controls (RMSSD <jats:italic>P<\/jats:italic>\u00a0=\u00a00.029); peri\u2010ictal values did not differ between the groups. Prolonged QRS\/PR was rare and more common in controls (QRS: one vs. none; PR: three vs. one).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>We did not identify major arrhythmias in DS which can directly explain high SUDEP rates. Peri\u2010ictal QTc\u2010lengthening was, however, more common in DS. This may reflect unstable repolarization and an\u00a0increased propensity for arrhythmias.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51017","type":"journal-article","created":{"date-parts":[[2020,3,24]],"date-time":"2020-03-24T08:40:51Z","timestamp":1585039251000},"page":"462-473","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Cardiac arrhythmias in Dravet syndrome: an observational multicenter study"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9413-6125","authenticated-orcid":false,"given":"Sharon","family":"Shmuely","sequence":"first","affiliation":[{"name":"Stichting Epilepsie Instellingen Nederland \u2010 SEIN Achterweg 5, 2103 SW Heemstede Dokter Denekampweg 20, 8025 BV Zwolle The Netherlands"},{"name":"NIHR University College London Hospitals Biomedical Research Centre UCL Queen Square Institute of Neurology Queen Square London WC1N 3BG UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rainer","family":"Surges","sequence":"additional","affiliation":[{"name":"Department of Epileptology University Hospital Bonn Bonn Germany"},{"name":"Centre for Rare Diseases Bonn (ZSEB) University Hospital Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert M.","family":"Helling","sequence":"additional","affiliation":[{"name":"Stichting Epilepsie Instellingen Nederland \u2010 SEIN Achterweg 5, 2103 SW Heemstede Dokter Denekampweg 20, 8025 BV Zwolle The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. Boudewijn","family":"Gunning","sequence":"additional","affiliation":[{"name":"Stichting Epilepsie Instellingen Nederland \u2010 SEIN Achterweg 5, 2103 SW Heemstede Dokter Denekampweg 20, 8025 BV Zwolle The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva H.","family":"Brilstra","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics University Medical Centre Utrecht Heidelberglaan 100 3584 CX Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith S.","family":"Verhoeven","sequence":"additional","affiliation":[{"name":"Academic Centre for Epileptology Kempenhaeghe 5590AB Heeze Heeze The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. 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