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QT and P wave dispersion and heart rate variability in patients with Dravet syndrome.
MedLine Citation:
PMID:  23065439     Owner:  NLM     Status:  Publisher    
SCN1A mutations are found in up to 80 % of patients with Dravet syndrome (DS), and the sudden unexpected death in epilepsy (SUDEP) rate is higher in DS than in most forms of severe epilepsy. The aim of this study is to examine the autonomic cardiac function and the risk of arrhythmia in DS patients by evaluating QT and P wave dispersion and heart rate variability (HRV) using standard electrocardiography (ECG) and 24-h ECG. The study group consisted of 15 patients (9 boys and 6 girls aged 3.5-17 years) who were genetically diagnosed with DS. The control group comprised 20 healthy subjects, 13 boys and 7 girls aged 4-17 years. P wave dispersion (44.6 ± 3.5 ms), QT dispersion (58.8 ± 7.5 ms) and QTc dispersion (70.8 ± 7.4 ms) were significantly higher in DS patients as compared to the control group (p < 0.001 for all values). However, there was no significant difference in PR, QT or QTc length between the groups. 24-h Holter ECG showed that all HRV parameters were significantly lower in patients with DS. The decreased HRV and increased P wave and QT dispersion seen in DS patients are important signs of autonomic dysfunction with increased adrenergic tone. To determine whether autonomic dysfunction is correlated with SUDEP in DS, long-term electrocardiographic monitoring and wider prospective studies are necessary.
Yakup Ergul; Baris Ekici; Burak Tatli; Kemal Nisli; Meral Ozmen
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-13
Journal Detail:
Title:  Acta neurologica Belgica     Volume:  -     ISSN:  0300-9009     ISO Abbreviation:  Acta Neurol Belg     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0247035     Medline TA:  Acta Neurol Belg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Department of Pediatric Cardiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey,
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