QT and P wave dispersion and heart rate variability in patients with Dravet syndrome
Tóm tắt
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.
Tài liệu tham khảo
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
Marini C, Mei D, Temudo T et al (2007) Idiopathic epilepsies with seizures precipitated by fever and SCN1A abnormalities. Epilepsia 48:1678–1685
Sakauchi M, Oguni H, Kato I et al (2011) Mortality in Dravet syndrome: search for risk factors in Japanese patients. Epilepsia 52:50–54
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
Nashef L, Hindocha N, Makoff A (2007) Risk factors in sudden death in epilepsy (SUDEP): the quest for mechanisms. Epilepsia 48:859–871
Stöllberger C, Finsterer J (2004) Cardiorespiratory findings in sudden unexplained/unexpected death in epilepsy (SUDEP). Epilepsy Res 59:51–60
Delogu AB, Spinelli A, Battaglia D et al (2011) Electrical and autonomic cardiac function in patients with Dravet syndrome. Epilepsia 52:55–58
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
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
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
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
Higham PD, Campbell RWF (1994) QT dispersion. Br Heart J 71:508–510
Akalin F, Tirtir A, Yilmaz Y (2003) Increased QT dispersion in epileptic children. Acta Paediatr 92:916–920
Davignon A, Rautaharju P, Boiselle E et al (1979) Normal ECG standards for infants and children. Pediatr Cardiol 1:123–131
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
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
Nabbout R (2008) Can SCN1A mutations account for SUDEP? Commentary on Hindocha et al. Epilepsia 49:367–368
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
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
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
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
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
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
Malik M, Batchvarov VN (2000) Measurement, interpretation and clinical potential of QT dispersion. J Am Coll Cardiol 36:1749–1766
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
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
Macfarlane PW, McLaughlin SC, Rodger JC (1998) Influence of lead selection and population on automated measurement of QT dispersion. Circulation 98:2160–2167
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
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
Nashef L, Ryvlin P (2009) Sudden unexpected death in epilepsy (SUDEP): update and reflections. Neurol Clin 27:1063–1074
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
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