Cardiac arrhythmias in Dravet syndrome: an observational multicenter study.

Autor: Shmuely S; Stichting Epilepsie Instellingen Nederland - SEIN, Achterweg 5, 2103 SW Heemstede, Dokter Denekampweg 20, 8025 BV, Zwolle, The Netherlands.; NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, Queen Square, London, WC1N 3BG, UK., Surges R; Department of Epileptology, University Hospital Bonn, Bonn, Germany.; Centre for Rare Diseases Bonn (ZSEB), University Hospital Bonn, Bonn, Germany., Helling RM; Stichting Epilepsie Instellingen Nederland - SEIN, Achterweg 5, 2103 SW Heemstede, Dokter Denekampweg 20, 8025 BV, Zwolle, The Netherlands., Gunning WB; Stichting Epilepsie Instellingen Nederland - SEIN, Achterweg 5, 2103 SW Heemstede, Dokter Denekampweg 20, 8025 BV, Zwolle, The Netherlands., Brilstra EH; Department of Medical Genetics, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands., Verhoeven JS; Academic Centre for Epileptology Kempenhaeghe, 5590AB Heeze, Heeze, The Netherlands., Cross JH; UCL NIHR BRC Great Ormond Street Institute of Child Health (ICH), 30 Guilford St, London, WC1N 1EH, UK., Sisodiya SM; NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, Queen Square, London, WC1N 3BG, UK.; Chalfont Centre for Epilepsy, Bucks, SL9 0RJ, UK., Tan HL; Heart Centre, Department of Experimental and Clinical Cardiology, Amsterdam University Medical Centres, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.; Netherlands Heart Institute, Moreelsepark 1, 3511 EP, Utrecht, The Netherlands., Sander JW; Stichting Epilepsie Instellingen Nederland - SEIN, Achterweg 5, 2103 SW Heemstede, Dokter Denekampweg 20, 8025 BV, Zwolle, The Netherlands.; NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, Queen Square, London, WC1N 3BG, UK.; Chalfont Centre for Epilepsy, Bucks, SL9 0RJ, UK., Thijs RD; Stichting Epilepsie Instellingen Nederland - SEIN, Achterweg 5, 2103 SW Heemstede, Dokter Denekampweg 20, 8025 BV, Zwolle, The Netherlands.; NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, Queen Square, London, WC1N 3BG, UK.; Department of Neurology, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
Jazyk: angličtina
Zdroj: Annals of clinical and translational neurology [Ann Clin Transl Neurol] 2020 Apr; Vol. 7 (4), pp. 462-473. Date of Electronic Publication: 2020 Mar 24.
DOI: 10.1002/acn3.51017
Abstrakt: Objectives: We ascertained the prevalence of ictal arrhythmias to explain the high rate of sudden unexpected death in epilepsy (SUDEP) in Dravet syndrome (DS).
Methods: We selected cases with clinical DS, ≥6 years, SCN1A mutation, and ≥1 seizure/week. Home-based ECG recordings were performed for 20 days continuously. Cases were matched for age and sex to two epilepsy controls with no DS and ≥1 major motor seizure during video-EEG. We determined the prevalence of peri-ictal 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.
Results: 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 (n = 11, 6.5%) than cases (n = 4, 0.7%; P = 0.002). Peri-ictal QTc-lengthening (≥60ms) occurred more frequently in DS (n = 64, 12%) than controls (n = 8, 4.7%, P = 0.048); pathologically prolonged QTc was rare (once in each group). In DS, interictal HRV was lower compared to controls (RMSSD P = 0.029); peri-ictal 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).
Interpretation: We did not identify major arrhythmias in DS which can directly explain high SUDEP rates. Peri-ictal QTc-lengthening was, however, more common in DS. This may reflect unstable repolarization and an increased propensity for arrhythmias.
(© 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.)
Databáze: MEDLINE
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