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. 2006 Oct 27;6(4):298–304. doi: 10.1111/j.1542-474X.2001.tb00122.x

Heart Rate Variability in Patients with Congenital Long QT Syndrome

Juha S Perkiömäki 1, Wojciech Zareba 1,✉, Jean‐Philippe Couderc 1, Arthur J Moss 1
PMCID: PMC7027695  PMID: 11686910

Abstract

Background: The congenital long QT syndrome (LQTS) affecting myocardial repolarization is caused by mutations in different cardiac potassium or sodium channel genes. Adrenergic triggers are known to initiate life‐threatening torsade de pointes ventricular tachycardias in LQTS patients, and anti‐adrenergic therapy has been shown to be effective in many cases. Despite this well‐documented adrenergic component, the data about autonomic modulation of the heart rate in LQTS, as described by heart rate variability (HRV) analysis, are very limited.

Methods: Conventional time‐ and frequency‐domain and newer nonlinear measures of HRV were compared in resting conditions among 27 LQTS patients with gene mutations at the LQT1 (n = 8), LQT2 (n = 10) or LQT3 (n = 9) loci and 34 LQTS noncarrier family members.

Results: None of the conventional time‐ or frequency‐domain or newer nonlinear measures of HRV differed significantly between the LQTS carriers and LQTS noncarriers or between the LQT1, LQT2, and LQT3 carriers.

Conclusions: These findings suggest that baseline cardiac autonomic modulation of the heart rate measured in resting conditions by traditional or newer nonlinear measures of HRV is not altered in LQTS patients. Furthermore, no differences are observed in HRV parameters between LQTS patients with potassium (KvLQT1, HERG), and sodium (SCN5A) ion channel gene mutations. HRV analysis in resting conditions does not improve phenotypic characterization of LQTS patients. A.N.E. 2001;6(4):298–304

Keywords: congenital long QT syndrome, heart rate variability

REFERENCES

  • 1. Keating M, Atkinson D, Dunn C, et al. Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras‐1 gene. Science 1991;252:704–706. [DOI] [PubMed] [Google Scholar]
  • 2. Jiang C, Atkinson D, Towbin JA, et al. Two long QT syndrome loci map to chromosome 3 and 7 with evidence for further heterogeneity. Nature Genet 1994;8:141–147. [DOI] [PubMed] [Google Scholar]
  • 3. Curran ME, Splawski I, Timothy KW, et al. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell 1995;80:795–803. [DOI] [PubMed] [Google Scholar]
  • 4. Schott JJ, Charpentier F, Peltier S, et al. Mapping of a gene for long QT syndrome to chromosome 4q25–27. Am J Hum Genet 1995;57:1114–1122. [PMC free article] [PubMed] [Google Scholar]
  • 5. Wang Q, Shen J, Splawski I, et al. SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell 1995;80:805–811. [DOI] [PubMed] [Google Scholar]
  • 6. Wang Q, Curran ME, Splawski I, et al. Positional cloning of a novel potassium channel gene: KvLQT1 mutations cause cardiac arrhythmias. Nat Genet 1996;12:17–23. [DOI] [PubMed] [Google Scholar]
  • 7. Schwartz PJ, Zaza A, Locati E, et al. Stress and sudden death: The case of the long QT syndrome. Circulation 1991;83(Suppl. H):II71–II80. [PubMed] [Google Scholar]
  • 8. Schwartz PJ, Locati E. The idiopathic long QT syndrome: Pathogenetic mechanisms and therapy. Eur Heart J 1985;6(Suppl. D):103–114. [DOI] [PubMed] [Google Scholar]
  • 9. Moss AJ, McDonald J. Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long QT interval syndrome. N Engl J Med 1971;285:903–904. [DOI] [PubMed] [Google Scholar]
  • 10. Schwartz PJ, Locati EH, Moss AJ, et al. Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome: A worldwide report. Circulation 1991;84:503–511. [DOI] [PubMed] [Google Scholar]
  • 11. Schwartz PJ, Periti M, Malliani A. The long Q‐T syndrome. Am Heart J 1975;89:378–390. [DOI] [PubMed] [Google Scholar]
  • 12. Mäkikallio TH, Seppänen T, Niemelä M, et al. Abnormalities in beat to beat complexity of heart rate dynamics in patients with a previous myocardial infarction. J Am Coll Cardiol 1996;28:1005–1011. [DOI] [PubMed] [Google Scholar]
  • 13. Mäkikallio TH, Seppänen T, Airaksinen KEJ, et al. Dynamic analysis of heart rate may predict subsequent ventricular tachycardia after myocardial infarction. Am J Cardiol 1997;80:779–783. [DOI] [PubMed] [Google Scholar]
  • 14. Huikuri HV, Niemelä MJ, Ojala S, et al. Circadian rhythms of frequency domain measures of heart rate variability in healthy subjects and patients with coronary artery disease. Circulation 1994;90:121–126. [DOI] [PubMed] [Google Scholar]
  • 15. Huikuri HV, Seppanen T, Koistinen MJ, et al. Abnormalities in beat‐to‐beat dynamics of heart rate before the spontaneous onset of life‐threatening ventricular tachyarrhythmias in patients with prior myocardial infarction. Circulation 1996;93:1836–1844. [DOI] [PubMed] [Google Scholar]
  • 16. Iyengar N, Peng CK, Morin R, et al. Age‐related alterations in the fractal scaling of cardiac interbeat interval dynamics. Am J Physiol 1996;271:R1078–R1084. [DOI] [PubMed] [Google Scholar]
  • 17. Peng CK, Havlin S, Stanley HE, et al. Quantification of scaling exponents and crossover phenomena in nonstationary heartbeat time series. CHAOS 1995;1:82–87. [DOI] [PubMed] [Google Scholar]
  • 18. Pincus SM. Approximate entropy as a measure of system complexity. Proc Natl Acad Sci USA 1991;88:2297–2301. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 19. Morita H, Yamanari H, Ohe T. Evaluation of autonomic nervous activity in patients with congenital long QT syndrome by an analysis of R‐R variability. Japan Circ J 1996;60:742–748. [DOI] [PubMed] [Google Scholar]
  • 20. Harrison TA, Mulroy MJ. Abnormal cardiac sensory innervation associated with experimentally induced, electrocardiographic long QT intervals in chick embryos. Pediatr Res 1996;39:90–97. [DOI] [PubMed] [Google Scholar]
  • 21. Zareba W, Moss AJ, Schwartz PJ, et al. for The International Long‐QT Syndrome Registry Research Group. Influence of the genotype on the clinical course of the long‐QT syndrome. N Engl J Med 1998;339: 960–965. [DOI] [PubMed] [Google Scholar]
  • 22. Hajj Ali RH, Zareba W, Moss AJ, et al. Clinical and genetic variables associated with acute arousal and non‐arousal‐related cardiac events among subjects with the long QT syndrome. Am J Cardiol 2000;85:457–461. [DOI] [PubMed] [Google Scholar]
  • 23. Pikkujämsä SM, Mäkikallio TH, Sourander LB, et al. Cardiac interbeat interval dynamics from childhood to senescence: Comparison of conventional and new measures based on fractals and chaos theory. Circulation 1999;100: 393–399. [DOI] [PubMed] [Google Scholar]
  • 24. Pitzalis MV, Mastropasqua F, Massari F, et al. Effects of hydrophilic and lipophilic beta‐blockers on heart rate variability and baroreflex sensitivity in normal subjects. PACE 1998;21:559–567. [DOI] [PubMed] [Google Scholar]
  • 25. Niemelä MJ, Airaksinen KEJ, Huikuri HV. Effect of beta‐blockade on heart rate variability in patients with coronary artery disease. J Am Coll Cardiol 1994;23:1370–1377. [DOI] [PubMed] [Google Scholar]

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