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Congenital Long QT Syndrome

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Cardiac Electrophysiology Review

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References

  1. Vincent GM. Long QT syndrome. Cardiology Clinics2000;18(2):309–325.

    Google Scholar 

  2. Piippo K, Swan H, Pasternack M, Chapman H, Paavonen K, Viitasalo M, Toivonen L, Kontula K. A founder mutation of the potassium channel KCNQ1 in long QT syndrome. Implications for estimation of disease prevalence and molecular diagnostics. J Am Coll Cardiol 2001;37:562–568.

    Google Scholar 

  3. Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, Denjoy I, Guicheney P, Breithardt G, Keating MT, Towbin JA, Beggs AH, Brink P, Wilde AAM, Toivonen L, Zareba W, Robinson J, Timothy KW, Corfield V, Wattanasirichaigoon D, Corbett C, Haverkamp W, Schulze-Bahr E, Lehmann MH, Schwartz K, Coumel P, Bloise R. Genotype-phenotype correlation in the long QT syndrome. Gene-specific triggers for life-threatening arrhythmias. Circulation2001;103:89–95.

    Google Scholar 

  4. Ackerman MJ, Tester DJ, Porter CJ. Swimming, a gene-specific arrhythmogenic trigger for inherited long QT syndrome. Mayo Clin Proc 1999;74: 1088–1094.

    Google Scholar 

  5. Wilde AAM, Jongbloed RJ, Doevendans PA, Duren DR, Hauer RN, van Langen IM, van Tintelen JP, Smeets HJ, Meyer H, Geelen JL. Auditory stimuli as a trigger for arrythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1). J Am Coll Cardiol1999;33:327–332.

    Google Scholar 

  6. Zareba W, Moss AJ, Schwartz PJ, Vincent GM, Robinson JL, Priori SG, Benhorin J, Locati EH, Towbin JA, Keating MT, Lehmann MH, Hall WJ. Influence of genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group. N Engl J Med1998;339:960–965.

    Google Scholar 

  7. Splawski I, Shen J, Timothy W, Lehmann MH, Priori S, Robinson JL, Moss AJ, Schwartz PJ, Towbin JA, Vincent GM, Keating MT. Spectrum of mutations in long-QT syndrome genes KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation2000;102:1178–1185.

    Google Scholar 

  8. Schulze-Bahr E, Wang Q, Wedekind H, Haverkamp W, Chen Q, Sun Y. KCNE1 mutations cause Jervell and Lange-Nielsen syndrome. Nat Genet 1997;17:267–268.

    Google Scholar 

  9. Splawski I, Timothy KW, Vincent GM, Atkinson DL, Keating MT. Molecular basis of the long-QT syndrome associated with deafness. Proc Assoc Am Physicians 1997;109:504–511.

    Google Scholar 

  10. Neyroud N, Tesson F, Denjoy I, Leibovici M, Donger C, Barhanin J, Faure S, Gary F, Coumel P, Petit C, Schwartz K, Guicheney P. A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome. Nat Genet 1997;15:186–189.

    Google Scholar 

  11. Shimizu W, Antzelevitch C. Sodium channel block with mexiletine is effective in reducing dispersion of repolarization and preventing torsade de pointes in LQT2 and LQT3 models of the long-QT syndrome. Circulation 1997;96:2038–2047.

    Google Scholar 

  12. Shimizu W, Antzelevitch C. Cellular basis for the ECG features of the LQT1 form of the long-QT syndrome: Effects of beta-adrenergic agonists and antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes. Circulation 1998;98:2314–2322.

    Google Scholar 

  13. Vincent GM, Timothy KW, Leppert M, Keating MT. The spectrum of symptoms and QT Intervals in the carriers of the gene for the long-QT syndrome. N Engl J Med 1992;327:846–852.

    Google Scholar 

  14. Vincent GM, Zhang L, Timothy KW, Fox J. Long QT syndrome patients with normal to borderline prolonged QTc intervals are at risk for syncope, cardiac arrest and sudden death. Circulation1999;100:1272–1272 (Abstract).

    Google Scholar 

  15. Zhang L, Timothy KW, Vincent GM, Lehmann MH, Fox J, Giuli LC, Shen J, Splawski I, Priori S, Compton SJ, Yanowitz F, Benhorin J, Moss AJ, Schwartz PJ, Robinson J, Wang Q, Zareba W, Keating M, Towbin JA, Napolitano C, Medina A. The spectrum of ST-T wave patterns and repolarization parameters in congenital long QT syndrome: ECG findings identify genotype. Circulation2000;102:2849–2855.

    Google Scholar 

  16. Lupoglazoff JM, Denjoy I, Berhet M, Neyroud N, Demay L, Richard P, Hainque B, Vaksmann G, Klug D, Leenhardt A, Maillard G, Coumel P, Guicheney P. Notched T waves on Holter recordings enhance detection of patients with LQT2 (HERG) mutations. Circulation2001;103:1095–1101.

    Google Scholar 

  17. Kaufman ES, Priori SG, NapolitanoC, Schwartz PJ, Iyengar S, Elston RC, Schnell AH, Gorodeski EZ, Rammohan G, Bahhur NO, Connuck D, Verrilli L, Rosenbaum DS, Brown AM. Electrocardiographic prediction of abnormal genotype in congenital long QT syndrome: Experience in 101 related family members. J Cardiovasc Electrophysiol 2001;12:455–461.

    Google Scholar 

  18. Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J, Vincent GM, Locati E, Priori SG, Napolitano C, Medina A, Zhang L, Robinson J, Timothy K, Towbin JA, Andrews ML. Effectiveness and limitations of beta-blocker therapy in congenital long QT syndrome. Circulation2000;101:616–623.

    Google Scholar 

  19. Viskin S. Cardiac pacing in the long QT syndrome: Review of available data and practical recommendations. J Cardiovasc Electrophysiol 2000;11: 593–600.

    Google Scholar 

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Authors and Affiliations

  1. Department of Medicine, LDS Hospital and University of Utah School of Medicine, 324 10th Avenue, Suite 130, Salt Lake City, UT, 84103

    G. Michael Vincent, Katherine Timothy & Li Zhang

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  1. G. Michael Vincent
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  2. Katherine Timothy
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  3. Li Zhang
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Vincent, G.M., Timothy, K. & Zhang, L. Congenital Long QT Syndrome. Card Electrophysiol Rev 6, 57–60 (2002). https://doi.org/10.1023/A:1017935121656

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