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QTc interval prolongation in children with Turner syndrome: the results of exercise testing and 24-h ECG

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Abstract

Background

Turner syndrome (TS) is the most common sex chromosome abnormality in females. Recently, a prolongation of the rate-corrected QT (QTc) interval in the electrocardiogram (ECG) of TS patients has been reported. A prolonged QTc interval has been correlated to an increased risk for sudden cardiac death, and medical treatment is warranted in patients with congenital long QT syndrome (LQTS). Additionally, several drugs of common use are contraindicated in LQTS because of their effects on myocardial repolarization. The importance of the QTc prolongation in TS patients is not known at present.

Materials and methods

Eighteen TS patients with a prolonged QTc interval (group 1) and 11 TS patients with a normal QTc interval (group 2) (mean age 12.6±3.1 vs. 11.8±2.1 years, respectively) were tested. The QTc interval was calculated during exercise testing and during 24-h ECG recordings.

Results

None of the patients experienced adverse cardiac events during the tests. The mean QTc interval decreased from 0.467 to 0.432 s in group 1 and from 0.432 to 0.412 s in group 2. During the 24-h ECG, the maximum QTc interval was significantly prolonged in group 1 (0.51 vs. 0.465 s, p<0.05, respectively). We conclude that exercise testing and 24-h ECG recording provide valuable information about the cardiac risk in the single TS patient with a prolonged QTc interval. This helps in counseling these girls, as clear therapeutic guidelines are currently lacking.

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References

  1. Algra A, Tijssen JG, Roelandt JR, Pool J, Lubsen J (1991) QTc prolongation measured by standard 12-lead electrocardiography is an independent risk factor for sudden death due to cardiac arrest. Circulation 83:1888–1894

    PubMed  CAS  Google Scholar 

  2. Bazett HC (1920) An analysis of the time relations of the electrocardiograms. Heart 7:353–370

    Google Scholar 

  3. Bondy CA; The Turner Syndrome Study Group (2007) Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab 92:10–25

    Article  PubMed  CAS  Google Scholar 

  4. Bondy CA, Ceniceros I, Van PL, Bakalov VK, Rosing DR (2006) Prolonged rate-corrected QT interval and other electrocardiogram abnormalities in girls with Turner syndrome. Pediatrics 118:1220–1225

    Article  Google Scholar 

  5. Bondy CA, Van PL, Bakalov VK, Sachdev V, Malone CA, Ho VB, Rosing DR (2006) Prolongation of the cardiac QTc interval in Turner syndrome. Medicine (Baltimore) 85:75–81

    Article  Google Scholar 

  6. Chiang CE (2004) Congenital and acquired long QT syndrome. Current concepts and management. Current concepts and management. Cardiol Rev 12:222–234

    Google Scholar 

  7. Crouch MA, Limon L, Cassano AT (2003) Clinical relevance and management of drug-related QT interval prolongation. Pharmacotherapy 23:881–908

    Article  PubMed  Google Scholar 

  8. Dalla Pozza R, Bechtold S, Kääb S, Buckl M, Urschel S, Netz H, Schwarz HP (2006) QTc interval prolongation in children with Ulrich-Turner syndrome. Eur J Pediatr 165:831–837

    Article  PubMed  Google Scholar 

  9. Dillenburg RF, Hamilton RM (2002) Is exercise testing useful in identifying congenital long QT syndrome? Am J Cardiol 89:233–236

    Article  PubMed  Google Scholar 

  10. Eggeling T, Osterhues HH, Hoeher M, Gabrielsen FG, Weismueller P, Hombach V (1992) Value of Holter monitoring in patients with the long QT syndrome. Cardiology 81:107–114

    Article  PubMed  CAS  Google Scholar 

  11. Garson A Jr (1993) How to measure the QT interval—what is normal? Am J Cardiol 72:14B–16B

    Article  PubMed  Google Scholar 

  12. Gravholt CH, Juul S, Naerraa RW, Hansen J (1998) Morbidity in Turner syndrome. J Clin Epidemiol 51:147–158

    Article  PubMed  CAS  Google Scholar 

  13. Gregoratos G, Abrams J, Epstein AE, Freedman RA, Hayes DL, Hlatky MA, Kerber RE, Naccarelli GV, Schoenfeld MH, Silka MJ, Winters SL, Gibbons RI, Antman EM, Alpert JS, Hiratzka LF, Faxon DP, Jacobs AK, Fuster V, Smith SC Jr; American College of Cardiology/American Heart Association Task Force on Practice Guidelines American College of Cardiology/American Heart Association/North American Society for Pacing and Electrophysiology Committee (2002) ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary article. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines). J Cardiovasc Electrophysiol 13:1183–1199

    Article  PubMed  Google Scholar 

  14. Li H, Fuentes-Garcia J, Towbin JA (2000) Current concepts in long QT syndrome. Pediatr Cardiol 21:542–550

    Article  PubMed  CAS  Google Scholar 

  15. Makarov LM, Belokoń NA, Laan MI, Belozerov YuM, Shkol’nikova MI, Krugliakov IV (1990) Holter monitoring in the long QT syndrome of children and adolescents. Cor Vasa 32:474–483

    PubMed  CAS  Google Scholar 

  16. Moss AJ, Schwartz PJ, Crampton RS, Locati E, Carleen E (1985) The long QT syndrome: a prospective international study. Circulation 71:17–21

    PubMed  CAS  Google Scholar 

  17. Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J, Vincent GM, Locati EH, Priori SG, Napolitano C, Medina A, Zhang L, Robinson JL, Timothy K, Towbin JA, Andrews ML (2000) Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 101:616–623

    PubMed  CAS  Google Scholar 

  18. Pearl W (1996) Effects of gender, age, and heart rate on QT intervals in children. Pediatr Cardiol 17:135–136

    Article  PubMed  CAS  Google Scholar 

  19. Price WH, Lauder IJ, Wilson J (1974) The electrocardiogram and sex chromosome aneuploidy. Clin Genet 6:1–14

    Article  PubMed  CAS  Google Scholar 

  20. Ranke MB, Saenger P (2001) Turner’s syndrome. Lancet 358:309–314

    Article  PubMed  CAS  Google Scholar 

  21. Rasche S, Koch T, Hübler M (2006) Long QT syndrome and anaesthesia. Anaesthesist 55:229–246

    Article  PubMed  CAS  Google Scholar 

  22. Roden DM (2004) Drug-induced prolongation of the QT interval. N Engl J Med 350:1013–1022

    Article  PubMed  CAS  Google Scholar 

  23. Roden DM, Temple R (2005) The US Food and Drug Administration Cardiorenal Advisory Panel and the drug approval process. Circulation 111:1697–1702

    Article  PubMed  Google Scholar 

  24. Saenger P (1996) Turner’s syndrome. N Engl J Med 335:1749–1754

    Article  PubMed  CAS  Google Scholar 

  25. Schouten EG, Dekker JM, Meppelink P, Kok FJ, Vandenbroucke JP, Pool J (1991) QT interval prolongation predicts cardiovascular mortality in an apparently healthy population. Circulation 84:1516–1523

    PubMed  CAS  Google Scholar 

  26. Schwartz PJ (2006) The congenital long QT syndromes from genotype to phenotype: clinical implications. J Intern Med 259:39–47

    Article  PubMed  CAS  Google Scholar 

  27. Swan H, Toivonen L, Viitasalo M (1998) Rate adaptation of QT intervals during and after exercise in children with congenital long QT syndrome. Eur Heart J 19:508–513

    Article  PubMed  CAS  Google Scholar 

  28. Vincent GM (1998) The molecular genetics of the long QT syndrome: genes causing fainting and sudden death. Annu Rev Med 49:263–274

    Article  PubMed  CAS  Google Scholar 

  29. Viskin S (1999) Long QT syndromes and torsade de pointes. Lancet 354:1625–1633

    Article  PubMed  CAS  Google Scholar 

  30. Walker BD, Krahn AD, Klein GJ, Skanes AC, Yee R (2005) Burst bicycle exercise facilitates diagnosis of latent long QT syndrome. Am Heart J 150:1059–1063

    Article  PubMed  Google Scholar 

  31. Webster R, Leishman D, Walker D (2002) Towards a drug concentration effect relationship for QT prolongation and torsades de pointes. Curr Opin Drug Discov Devel 5:116–126

    PubMed  CAS  Google Scholar 

  32. Yap YG, Camm AJ (2003) Drug induced QT prolongation and torsades de pointes. Heart 89:1363–1372

    Article  PubMed  CAS  Google Scholar 

  33. Zareba W, Moss AJ, Daubert JP, Hall WJ, Robinson JL, Andrews M (2003) Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol 14:337–341

    Article  PubMed  Google Scholar 

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Correspondence to Robert Dalla Pozza.

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Dalla Pozza, R., Bechtold, S., Urschel, S. et al. QTc interval prolongation in children with Turner syndrome: the results of exercise testing and 24-h ECG. Eur J Pediatr 168, 59–64 (2009). https://doi.org/10.1007/s00431-008-0709-y

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  • DOI: https://doi.org/10.1007/s00431-008-0709-y

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