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Gene Therapy for Long QT Syndrome: Fact or Fiction?

  • Conference paper
Cardiac Arrhythmias 1999
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Abstract

For many years the therapy for long QT syndrome (LQTS) has been based on simple and sound principles [1, 2]. In essence, therapy had been guided by the time-honored concept that it has to reflect understanding of the mechanisms, underlying or precipitating the main symptoms. In those happy days things were simple, we thought that we were dealing with one disease, and we were assuming that the precipitating events were more or less the same for all patients; the gen- erally accepted common pathway for the initiation of the life-threatening arrhythmias of LQTS was represented by a sudden neural release of norepineph- rine secondary to an abrupt activation of the sympathetic nervous system [2].

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References

  1. Schwartz PJ, Periti M, Malliani A (1975) The long Q-T syndrome. Am Heart J 89:378–390

    Article  PubMed  CAS  Google Scholar 

  2. Schwartz PJ (1985) Idiopathic long QT syndrome: progress and questions. Am Heart J 109:399–411

    Article  PubMed  CAS  Google Scholar 

  3. Moss AJ, McDonald J (1971) Unilateral cervicothoracic sympathetic ganglionec-tomy for the treatment of long QT interval syndrome. N Engl J Med 285:903–904

    Article  PubMed  CAS  Google Scholar 

  4. Schwartz PJ, Locati EH, Moss AJ, Crampton RS, Trazzi R, Ruberti U (1991) Left car-diac sympathetic denervation in the therapy of congenital long QT syndrome: a worldwide report. Circulation 84:503–511

    Article  PubMed  CAS  Google Scholar 

  5. Roden DM, Lazzara R, Rosen MR, Schwartz PJ, Towbin JA, Vincent GM, for the SADS Foundation Task Force on LQTS (1996) Multiple mechanisms in the long-QT syndrome. Current knowledge, gaps, and future directions. Circulation 94:1996– 2012

    CAS  Google Scholar 

  6. Priori SG, Barhanin J, Hauer RNW, Haverkamp W, Jongsma HJ, Kleber AG, McKenna WJ, Roden DM, Rudy Y, Schwartz K, Schwartz PJ, Towbin JA, Wilde AM (1999) Genetic and molecular basis of cardiac arrhythmias: impact on clinical management. Part I and II. Circulation 99:518-528; Part III. Circulation 99:674–681

    CAS  Google Scholar 

  7. Schwartz PJ, Priori SG, Locati EH, Napolitano C, Cantù F, Towbin AJ, Keating MT, Hammoude H, Brown AM, Chen LK, Colatsky TJ (1995) Long QT syndrome patients with mutations on the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-spe-cific therapy. Circulation 92:3381–3386

    CAS  Google Scholar 

  8. Bennett PB, Yazawa K, Makita N, George AL Jr (1995) Molecular mechanism for an inherited cardiac arrhythmia. Nature 376:683–685

    Article  PubMed  CAS  Google Scholar 

  9. Dumaine R, Wang Q, Keating MT, Hartmann HA, Schwartz PJ, Brown AM, Kirsch GE (1996) Multiple mechanisms of Na+ channel-linked long-QT syndrome. Circ Res 78:916–924

    Article  PubMed  CAS  Google Scholar 

  10. Rosero SZ, Zareba W, Robinson JL, Moss AJ (1997) Gene-specifìc therapy for long QT syndrome: QT shortening with lidocaine and tocainide in patients with muta-tion of the sodium channel gene. ANE 3:274–278

    Google Scholar 

  11. An RH, Wang XL, Kerem B, Benhorin J, Medina A, Goldmit M, Kass RS (1998) Novel LQT-3 mutation affects Na+ channel activity through interactions between a-and β1-subunits. Circ Res 83:141–146

    Article  Google Scholar 

  12. Compton SJ, Lux RL, Ramsey MR, Strelich KT, Sanguinetti MC, Green LS, Keating MT, Mason JW (1996) Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium. Circulation 94:1018–1022

    CAS  Google Scholar 

  13. Sanguinetti MC, Curran ME, Spector PS, Keating MT (1996) Spectrum of HERG K+-channel dysfunction in an inherited cardiac arrhythmia. Proc Natl Acad Sci USA 93:2208–2212

    Article  PubMed  CAS  Google Scholar 

  14. Tan HL, Alings M, Van Olden RW, Wilde AAM (1999) Long-term (subacute) potas-sium treatment in congenital HERG-related long QT syndrome (LQTS2). J Cardiovasc Electrophysiol 10:229–233

    Article  PubMed  CAS  Google Scholar 

  15. Schwartz PJ (1997) The long QT syndrome. In: Camm AJ (ed) Clinical approaches to tachyarrhythmias series. Futura, Armonk, NY

    Google Scholar 

  16. Schwartz PJ, Priori SG, Napolitano C. Long QT syndrome. In: Zipes DP, Jalife J (eds) Cardiac electrophysiology. From cell to bedside, 3rd edn. WB Saunders, Philadelphia (in press)

    Google Scholar 

  17. Schwartz PJ, Moss AJ, Priori SG, Wang Q, Lehmann MH, Timothy K, Denjoy I, Haverkamp W, Guicheney P, Paganini V, Scheinman MM, Karnes PS (1997) Gene-specific influence on the triggers for cardiac arrest in the long QT syndrome. Circulation 96[Suppl]:212 (abstr)

    Article  Google Scholar 

  18. Priori SG, Napolitano C, Locati EH, Cantù F, Stramba-Badiale M, Keating MT, Towbin JA, Colatsky TJ, Chen LK, Schwartz PJ (1996) Long QT syndrome patients genetically linked to defective genes on chromosomes 11, 7 and 3 present differential response to changes in heart rate. J Am Coll Cardiol 27[Suppl A]:171A

    Article  Google Scholar 

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© 2000 Springer-Verlag Italia

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Schwartz, P.J. (2000). Gene Therapy for Long QT Syndrome: Fact or Fiction?. In: Raviele, A. (eds) Cardiac Arrhythmias 1999. Springer, Milano. https://doi.org/10.1007/978-88-470-2139-6_40

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  • DOI: https://doi.org/10.1007/978-88-470-2139-6_40

  • Publisher Name: Springer, Milano

  • Print ISBN: 978-88-470-2178-5

  • Online ISBN: 978-88-470-2139-6

  • eBook Packages: Springer Book Archive

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