Skip to main content

Advertisement

SpringerLink
Log in

QT and P wave dispersion and heart rate variability in patients with Dravet syndrome

  • Original Article
  • Published:
Acta Neurologica Belgica Aims and scope Submit manuscript

Abstract

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Dravet C, Bureau M, Oguni H, Fukuyama Y, Cokar O (2005) Severe myoclonic epilepsy in infancy (Dravet syndrome). In: Roger J, Bureau M, Dravet C, Genton P, Tassinari CA, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence, 4th edn. John Libbey, London, pp 77–89

    Google Scholar 

  2. Marini C, Mei D, Temudo T et al (2007) Idiopathic epilepsies with seizures precipitated by fever and SCN1A abnormalities. Epilepsia 48:1678–1685

    Article  PubMed  CAS  Google Scholar 

  3. Sakauchi M, Oguni H, Kato I et al (2011) Mortality in Dravet syndrome: search for risk factors in Japanese patients. Epilepsia 52:50–54

    Article  PubMed  Google Scholar 

  4. Hitiris N, Suratman S, Kelly K, Stephen LJ, Sills GJ, Brodie MJ (2007) Sudden unexpected death in epilepsy: a search for risk factors. Epilepsy Behav 10:138–141

    Article  PubMed  Google Scholar 

  5. Nashef L, Hindocha N, Makoff A (2007) Risk factors in sudden death in epilepsy (SUDEP): the quest for mechanisms. Epilepsia 48:859–871

    Article  PubMed  Google Scholar 

  6. Stöllberger C, Finsterer J (2004) Cardiorespiratory findings in sudden unexplained/unexpected death in epilepsy (SUDEP). Epilepsy Res 59:51–60

    Article  PubMed  Google Scholar 

  7. Delogu AB, Spinelli A, Battaglia D et al (2011) Electrical and autonomic cardiac function in patients with Dravet syndrome. Epilepsia 52:55–58

    Article  PubMed  Google Scholar 

  8. Hindocha N, Nashef L, Elmslie F et al (2008) Two cases of sudden unexpected death in epilepsy in a GEFS+ family with an SCN1A mutation. Epilepsia 49:360–365

    Article  PubMed  Google Scholar 

  9. Maier SK, Westenbroek RE, Yamanushi TT et al (2003) An unexpected requirement for brain-type sodium channels for control of heart rate in the mouse sinoatrial node. Proc Natl Acad Sci USA 100:3507–3512

    Article  PubMed  CAS  Google Scholar 

  10. Kanter RJ (1998) Syncope and sudden death. In: Garson A Jr, Bricker JT, Fisher DJ et al (eds) The science and practice of pediatric cardiology. Williams & Wilkins, Baltimore, pp 2169–2199

    Google Scholar 

  11. Babaoglu K, Altun G, Binnetoğlu K (2011) P-wave dispersion and heart rate variability in children with mitral valve prolapse. Pediatr Cardiol 32:449–454

    Article  PubMed  Google Scholar 

  12. Higham PD, Campbell RWF (1994) QT dispersion. Br Heart J 71:508–510

    Article  PubMed  CAS  Google Scholar 

  13. Akalin F, Tirtir A, Yilmaz Y (2003) Increased QT dispersion in epileptic children. Acta Paediatr 92:916–920

    Article  PubMed  CAS  Google Scholar 

  14. Davignon A, Rautaharju P, Boiselle E et al (1979) Normal ECG standards for infants and children. Pediatr Cardiol 1:123–131

    Article  Google Scholar 

  15. American College of Cardiology/American Heart Association (1998) ACC/AHA guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients with Valvular Heart Disease). J Am Coll Cardiol 32:1486–1588

    Article  Google Scholar 

  16. Monté CP, Arends JB, Tan IY, Aldenkamp AP, Limburg M, de Krom MC (2007) Sudden unexpected death in epilepsy patients: risk factors. A systematic review. Seizure 16:1–7

    Article  PubMed  Google Scholar 

  17. Nabbout R (2008) Can SCN1A mutations account for SUDEP? Commentary on Hindocha et al. Epilepsia 49:367–368

    Article  PubMed  Google Scholar 

  18. Johnson JN, Hofman N, Haglund CM, Cascino GD, Wilde AAM, Ackerman MJ (2009) Identification of a possible pathogenic link between congenital long QT syndrome and epilepsy. Neurology 72:224–231

    Article  PubMed  CAS  Google Scholar 

  19. Lei M, Jones SA, Liu J et al (2004) Requirement of neuronal- and cardiac-type sodium channels for murine sinoatrial node pacemaking. J Physiol 559:835–848

    PubMed  CAS  Google Scholar 

  20. Li Z, Hertervig E, Carlson J, Johansson C, Olsson SB, Yuan S (2002) Dispersion of refractoriness in patients with paroxysmal atrial fibrillation. Evaluation with simultaneous endocardial recordings from both atria. J Electrocardiol 35:227–234

    Article  PubMed  Google Scholar 

  21. Day CP, McComp LM, Campbell RWF (1990) QT dispersion: an indication of arrhythmia risk in patients with long QT intervals. Br Heart J 63:342–344

    Article  PubMed  CAS  Google Scholar 

  22. Cheema AN, Ahmed MW, Kadish AH, Goldberger JJ (1995) Effects of autonomic stimulation and blockade on signal-averaged P wave duration. J Am Coll Cardiol 26:497–502

    Article  PubMed  CAS  Google Scholar 

  23. Guven B, Eroglu AG, Babaoglu K et al (2008) QT dispersion and diastolic functions in differential diagnosis of primary mitral valve prolapse and rheumatic mitral valve prolapse. Pediatr Cardiol 29:352–358

    Article  PubMed  Google Scholar 

  24. Malik M, Batchvarov VN (2000) Measurement, interpretation and clinical potential of QT dispersion. J Am Coll Cardiol 36:1749–1766

    Article  PubMed  CAS  Google Scholar 

  25. Engel G, Beckerman JG, Froelicher VF, Yamazaki T, Chen HA, Richardson K, McAuley RJ, Ashley EA, Chun S, Wang PJ (2004) Electrocardiographic arrhythmia risk testing. Curr Probl Cardiol 29:365–432

    Article  PubMed  Google Scholar 

  26. Surges R, Taggart P, Sander JW, Walker MC (2010) Too long or too short? New insights into abnormal cardiac repolarization in people with chronic epilepsy and its potential role in sudden unexpected death. Epilepsia 51:738–744

    Article  PubMed  Google Scholar 

  27. Macfarlane PW, McLaughlin SC, Rodger JC (1998) Influence of lead selection and population on automated measurement of QT dispersion. Circulation 98:2160–2167

    Article  PubMed  CAS  Google Scholar 

  28. Rasmussen KG, Hooten WM, Dodd ML, Ryan DA (2007) QTc dispersion on the baseline ECG predicts arrhythmias during electroconvulsive therapy. Acta Cardiol 62:345–347

    Article  PubMed  Google Scholar 

  29. Köken R, Demir T, Sen TA, Kundak AA, Oztekin O, Alpay F (2010) The relationship between P-wave dispersion and diastolic functions in diabetic children. Cardiol Young 20:133–137

    Article  PubMed  Google Scholar 

  30. Nashef L, Ryvlin P (2009) Sudden unexpected death in epilepsy (SUDEP): update and reflections. Neurol Clin 27:1063–1074

    Article  PubMed  Google Scholar 

  31. Junttila MJ, Gonzalez M, Lizotte E, Benito B, Vernooy K, Sarkozy A, Huikuri HV, Brugada P, Brugada J, Brugada R (2008) Induced Brugada-type electrocardiogram, a sign for imminent malignant arrhythmias. Circulation 117:1890–1893

    Article  PubMed  Google Scholar 

  32. Surges R, Adjei P, Kallis C, Erhuero J, Scott CA, Bell GS, Sander JW, Walker MC (2010) Pathologic cardiac repolarization in pharmacoresistant epilepsy and its potential role in sudden unexpected death in epilepsy: a case–control study. Epilepsia 51:233–242

    Article  PubMed  Google Scholar 

Download references

Conflicts of interest

The authors report no conflicts of interest.

Author information

Authors and Affiliations

  1. Department of Pediatric Cardiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

    Yakup Ergul & Kemal Nisli

  2. Department of Pediatric Neurology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey

    Baris Ekici & Meral Ozmen

  3. Arkeon sitesi A5 blok D.3 Dereboyu Cad. Ortakoy/Beşiktaş, Istanbul, Turkey

    Baris Ekici

  4. Department of Pediatric Neurology, Bezmialem Vakıf University, Istanbul, Turkey

    Burak Tatli

Authors
  1. Yakup Ergul
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Baris Ekici
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Burak Tatli
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Kemal Nisli
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Meral Ozmen
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Baris Ekici.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ergul, Y., Ekici, B., Tatli, B. et al. QT and P wave dispersion and heart rate variability in patients with Dravet syndrome. Acta Neurol Belg 113, 161–166 (2013). https://doi.org/10.1007/s13760-012-0140-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13760-012-0140-z

Keywords

Search

Navigation