Cardiac arrhythmias in Dravet syndrome: an observational multicenter study

Autor:	W. Boudewijn Gunning, Sharon Shmuely, Roland D. Thijs, Sanjay M. Sisodiya, Judith S. Verhoeven, Rainer Surges, Hanno L. Tan, Eva H. Brilstra, Robert M. Helling, Josemir W. Sander, J. Helen Cross
Přispěvatelé:	Cardiology, ACS - Heart failure & arrhythmias, APH - Methodology
Rok vydání:	2020
Předmět:	Adult Male 0301 basic medicine Bradycardia medicine.medical_specialty Adolescent Epilepsies Myoclonic Neurosciences. Biological psychiatry. Neuropsychiatry QT interval Electrocardiography Young Adult 03 medical and health sciences QRS complex Epilepsy 0302 clinical medicine Dravet syndrome Internal medicine Heart rate Prevalence medicine Humans Ictal cardiovascular diseases Sudden Unexpected Death in Epilepsy Asystole Child RC346-429 Research Articles business.industry General Neuroscience Arrhythmias Cardiac Electroencephalography medicine.disease 030104 developmental biology Cardiology Female Neurology. Diseases of the nervous system Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery Research Article RC321-571
Zdroj:	Annals of Clinical and Translational Neurology, 7(4), 462-473. WILEY Annals of Clinical and Translational Neurology, Vol 7, Iss 4, Pp 462-473 (2020) Annals of Clinical and Translational Neurology Annals of clinical and translational neurology, 7(4), 462-473. John Wiley and Sons Ltd
ISSN:	2328-9503
Popis:	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.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::652495b6824a731aab31c3d11d4e38af https://doi.org/10.1002/acn3.51017 Zobrazit plný text záznamu Plný text ve formátu PDF Plný text ve formátu HTML
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