Magnetocardiographic Imaging of Ventricular Repolarization in Rett Syndrome

  • Donatella Brisinda
  • Anna Maria Meloni
  • Giuseppe Hayek
  • Menotti Calvani
  • Riccardo Fenici
Conference paper
Part of the Lecture Notes in Computer Science book series (LNCS, volume 3504)

Abstract

Rett syndrome (RS) is a severe neurological disorder, predominant in females, with higher risk of sudden death (SD). So far for risk-assessment, heart rate variability (HRV), QT duration and its dispersion (QTd) were measured with ECG. However SD has occurred in RS also in absence of ECG abnormality. We aimed to evaluate the feasibility of magnetocardiographic (MCG) mapping as an alternative to study ventricular repolarization (VR) alteration in RS patients. 9 female (age: 1-34 years) RS patients were studied with an unshielded 36-channels MCG system. To assess VR, heart rate (HR)-corrected JTpeak, JTend, QTend, Tpeak − − endintervals and QTd, were measured from both MCG and ECG signals. Moreover the magnetic field (MF) gradient orientation (α-angle) during the ST segment and three MF dynamic parameters were automatically evaluated from MCG T-wave. HRV parameters were evaluated from 12-lead Holter ECG. 15 age-matched normal controls (NC) were studied for comparison. HR-corrected JTpeak, JTend, QTend and Tpeak − − end intervals, and QTd were longer in RS than in NC. The differences were more evident with clinical impairment (stage IV). MF gradient orientation and MF dynamic parameters were abnormal in RS patients. As compared to NC, HRV parameters were altered in the time-domain, although still within normal range in the frequency-domain. In RS, ECG recordings are often noisy and BSPM is difficult. On the contrary MCG mapping is easily feasible and discovers VR alteration not evident at the ECG. The diagnostic value of MCG in RS remains to be defined.

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References

  1. 1.
    Jellinger, K.A.: Rett syndrome- an update. J. Neural Transm. 110, 681–701 (2003)CrossRefGoogle Scholar
  2. 2.
    Dunn, H.G., MacLeod, P.M.: Rett syndrome: review of biological abnormalities. Can. J. Neurol. Sci. 28(1), 16–29 (2001)Google Scholar
  3. 3.
    Rosenberg, C., Wouters, C.H., Szuhai, K., Dorland, R., Pearson, P., Tien Poll-The, B., Colombijn, R.M., Breuning, M., Lindhout, D.: A Rett syndrome patient with a ring X chromosome: further evidence for skewing of X inactivation and heterogeneity in the aetiology of the disease. Eur. J. Hum. Genet. 9(3), 171–177 (2001)CrossRefGoogle Scholar
  4. 4.
    Hagberg, B.A., Witt-Engerstrom, I.: Rett Syndrome: A suggested staging system for describing impairment profile with increasing age towards adolescence. American J. Med. genetics 24, 47–59 (1986)CrossRefGoogle Scholar
  5. 5.
    Trevathan, F.: The Rett syndrome Diagnosis Criteria Working Group. Diagnostic criteria for Rett syndrome. Ann. Neurol. 23, 425–428 (1988)CrossRefGoogle Scholar
  6. 6.
    Naidu, S.: Rett syndrome. A disorder affecting early brain growth. Ann. Neurol. 42(1), 3–10 (1997)CrossRefMathSciNetGoogle Scholar
  7. 7.
    Kerr, A.M., Armstrong, D.D., Prescott, R.J., Doyle, D., Kearney, D.L.: Rett syndrome: analysis of deaths in the British survey. Eur Child Adolesc Psychiatry 6(suppl. 1), 71–74 (1997)Google Scholar
  8. 8.
    Driscoll, D.J., Edwards, W.D.: Sudden unexpected death in children and adolescents. J. Am. Coll. Cardiol. 5(6 Suppl), 118B–121B (1985)CrossRefGoogle Scholar
  9. 9.
    Julu, P., Kerr, A.M., Apartopoulos, F., Alrawas, S., Witt Engerstrom, I., Jamal, G.A., Hansen, S.: Characterization of breathing and associated central autonomic dysfunction in the Rett disorder. Arch. Dis. Child. 85, 29–37 (2001)CrossRefGoogle Scholar
  10. 10.
    Wenk, G.L., Hauss-Wegrzyniak, B.: Altered cholinergic function in the basal forebrain of girls with Rett syndrome. Neuropediatrics 30(3), 125–129 (1999)CrossRefGoogle Scholar
  11. 11.
    Guideri, F., Acampa, M., Calamandrei, G., Aloe, L., Zappella, M., Hayek, Y.: Nerve Growth Factor Plasma Levels and Ventricular Repolarization in Rett Syndrome. Pediatr. Cardiol. 25(4), 394–396 (2004)CrossRefGoogle Scholar
  12. 12.
    Sekul, E.A., Moak, J.P., Schultz, R.J., Glaze, D., Dunn, J.K., Percy, A.K.: Electrocardiographic findings in Rett Syndrome: an explanation for sudden death? The Journal of Pediatrics 125, 80–82 (1994)CrossRefGoogle Scholar
  13. 13.
    Ellaway, C.J., Sholler, G., Leonard, H., Christodoulou, J.: Prolonged QT interval in Rett syndrome. Arch. Dis. Child. 80, 470–472 (1999)CrossRefGoogle Scholar
  14. 14.
    Guideri, F., Acampa, M., Hayek, G., Zappella, M., Di Perri, T.: Reduced heart rate variability in patients affected with Rett sindrome. A possible explanation of sudden death. Neuropediatrics 30, 146–148 (1999)CrossRefGoogle Scholar
  15. 15.
    Guideri, F., Acampa, M., Di Perri, T., Zappella, M., Hayek, Y.: Progressive cardiac disautonomia observed in patients affected by classic Rett syndrome and not preserved speech variant. J. Child Neurol. 16, 370–373 (2001)CrossRefGoogle Scholar
  16. 16.
    Dearlove, O.R., Walker, R.W.M.: Anesthesia for Rett syndrome. Pediatr. Anaesth. 6, 155–158 (1996)CrossRefGoogle Scholar
  17. 17.
    Tavarozzi, I., Comani, S., Del Gatta, C., Di Luzio, S., Romani, G.L., Gallina, S., Zimarino, M., Brisinda, D., Fenici, R., De Caterina, R.: Magnetocardiography: current status and perspectives. Part II: Clinical applications. Italian Heart J. 3(2), 151–165 (2002)Google Scholar
  18. 18.
    Wikswo, J.P., Barach, J.: Possible sources of new information in the magnetocardiogram. Journal of Theoretical Biology 95, 721–729 (1982)CrossRefGoogle Scholar
  19. 19.
    Brockmeier, K., Schmitz, L., Bobadilla Chavez, J.D., Burghoff, M., Koch, H., Zimmermann, R., Trahms, L.: Magnetocardiography and 32-lead potential mapping: repolarization in normal subjects during pharmacologically induced stress. J. Cardiovasc. Electrophysiol. 18, 615–626 (1997)CrossRefGoogle Scholar
  20. 20.
    Korhonen, P., Väänanen, H., Mäkijärvi, M., Katila, T., Toivonen, L.: Repolarization abnormalities detected by magnetocardiography in patients with dilated cardiomyopathy and ventricular arrhythmias. J. Cardiovasc. Electrophysio. 12, 772–777 (2001)CrossRefGoogle Scholar
  21. 21.
    Korhonen, P., Pesola, K., Jarvinen, A., Makijarvi, M., Katila, T., Toivonen, L.: Relation of magnetocardiographic arrhythmia risk parameters to delayed ventricular conduction in postinfarction ventricular tachycardia. Pacing Clin. Electrophysiol. 25(9), 1339–1345 (2002)CrossRefGoogle Scholar
  22. 22.
    Fenici, R., Brisinda, D., Meloni, A.M., Fenici, P.: First 36-channel System for Clinical Magnetocardiography in Unshielded Hospital Laboratory for Cardiac Electrophysiology. International Journal of Bioelectromagnetism 5(1), 80–83 (2003)Google Scholar
  23. 23.
    Task force of the European Society of Cardiology and the North American Society of Pacing and Electrophysiology. Heart Rate Variability standards of measurement, physiological interpretation and clinical use. Circulation 93, 1043–1065 (1996)Google Scholar
  24. 24.
    Brisinda, D., Meloni, A.M., Fenici, R.: First 36-channel Magnetocardiographic Study of CAD Patients in an Unshielded Laboratory for Interventional and Intensive Cardiac Care. In: Magnin, I.E., Montagnat, J., Clarysse, P., Nenonen, J., Katila, T. (eds.) FIMH 2003. LNCS, vol. 2674, pp. 122–131. Springer, Heidelberg (2003)CrossRefGoogle Scholar
  25. 25.
    Brisinda, D., Meloni, A.M., Fenici, P., Fenici, R.: Unshielded Multichannel Magnetocardiographic Study of Ventricular Repolarization in Healthy Subjects. Biomed. Tech. 48(2), 165–167 (2004)Google Scholar
  26. 26.
    Hänninen, H., Takala, P., Mäkijärvi, M., Montonen, J., Korhronen, P., Oikarinen, L., Nenonen, J., Katila, T., Toivonen, L.: Detection of exercise induced myocardial ischemia by multichannel magnetocardiography in patients with single vessel coronary artery disease. Ann. Noninv. Electrocardiology 5, 147–157 (2000)CrossRefGoogle Scholar
  27. 27.
    Goto, M., Nagashima, M., Baba, R., Nagano, Y., Yokota, M., Nishibata, K., Tsuji, A.: Analysis of heart rate variability demonstrates effects of development on vagal modulation of heart rate in healthy children. The Journal of pediatrics 130(5), 725–729 (1997)CrossRefGoogle Scholar
  28. 28.
    Umetani, K., Singer, D., McCraty, R., Atkinson, M.: Twenty-four hour time domain heart rate variability and heart rate: relations to age and gender over nine decades. J. Am. Coll. Cardiol. 31, 593–601 (1998)CrossRefGoogle Scholar
  29. 29.
    Kardys, I., Kors, J.A., van der Meer, I.M., Hofman, A., van der Kuip, D.A., Witteman, J.C.: Spatial QRS-T angle predicts cardiac death in a general population. Eur. Heart J. 24, 1357–1364 (2003)CrossRefGoogle Scholar
  30. 30.
    Kannel, W.B., Anderson, K., McGee, D.L., Degatano, L.S., Stampfer, M.J.: Non-specific electrocardiographic abnormality as a predictor of coronary heart disease. The Framingham Study. Am. Heart J. 113, 370–376 (1987)CrossRefGoogle Scholar
  31. 31.
    de Bruyne, M.C., Hoes, A.W., Kors, J.A., Hofman, A., van Bemmel, J.H., Grobbee, D.E.: Prolonged QT interval predicts cardiac and all-cause mortality in the elderly. The Rotterdam Study. Eur. Heart J. 20, 278–284 (1999)CrossRefGoogle Scholar
  32. 32.
    Yan, G.X., Antzelevitch, C.: Cellular basis for the normal T wave and Electrocardiographic manifestation of the Long-QT syndrome. Circulation 98, 1928–1936 (1998)Google Scholar
  33. 33.
    Oikarinen, L., Viitasalo, M., Korhonen, P., Vaananen, H., Hanninen, H., Montonen, J., Makijarvi, M., Katila, T., Toivonen, L.: Postmyocardial infarction patients susceptible to ventricular tachycardia show increased T wave dispersion independent of delayed ventricular conduction. J. Cardiovasc. Electrophysiol. 12, 1115–1120 (2001)CrossRefGoogle Scholar
  34. 34.
    Steinberg, B.A., Roguin, A., Allen, E., Wahl, D.R., Smith, C.S., St John, M.: Reproducibility and interpretation of MCG maps in detecting ischemia. In: Personal Communication, ACC (March 2004)Google Scholar
  35. 35.
    Fenici, R., Bison, G., Wynands, R., Brisinda, D., Meloni, A.M., Weis, A.: Comparison of Magnetocardiographic Mapping with SQUID-based and Laser-pumped Magnetometers in Normal Subjects. Biomed. Tech. 48(suppl. 2), 192–194 (2004)Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2005

Authors and Affiliations

  • Donatella Brisinda
    • 1
  • Anna Maria Meloni
    • 1
  • Giuseppe Hayek
    • 2
  • Menotti Calvani
    • 3
  • Riccardo Fenici
    • 1
  1. 1.Clinical Physiology–Biomagnetism CenterCatholic University of RomeItaly
  2. 2.Department of Child Neurology and PsychiatryUniversity of SienaItaly
  3. 3.Sigma Tau S.p.A.RomeItaly

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