Skip to main content
SpringerLink
Log in

Channellopathies: New ECG Criteria for Risk Stratification

  • Chapter
  • First Online:
New Concepts in ECG Interpretation

  • 1917 Accesses

Abstract

Cardiac channellopathies—such as long QT syndrome (LQTS), Brugada syndrome (BrS), and short QT syndrome (SQTS)—are genetic disorders secondary to different ion channel mutations predisposing to ventricular tachyarrhythmias and sudden cardiac death (SCD). Arrhythmic risk is particularly elevated in symptomatic patients, and nonfatal events (syncope, documented TV, and aborted cardiac arrest) remain the strongest predictor of SCD and are an indication to ICD implantation.

Some marker and specific finding on the basal electrocardiogram (such as QT length and dispersion in LQTS or the association with atrial fibrillation and conduction delays in Brugada syndrome) can help in estimating the specific arrhythmic risk.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 99.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Calò L, Giustetto C, Martino A, et al. A new electrocardiographic marker of sudden death in brugada syndrome: The S-wave in lead I. J Am Coll Cardiol. 2016;67(12):1427–40.

    Article  Google Scholar 

  2. Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005;111(5):659–70.

    Article  Google Scholar 

  3. Fowler SJ, Priori SG. Clinical spectrum of patients with a Brugada ECG. Curr Opin Cardiol. 2009;24(1):74–81.

    Article  Google Scholar 

  4. Kapplinger JD, Tester DJ, Alders M, et al. An international compendium of mutations in the SCN5A-encoded cardiac sodium channel in patients referred for Brugada syndrome genetic testing. Heart Rhythm. 2010;7(1):33–46.

    Article  Google Scholar 

  5. Antzelevitch C, Patocskai B. Brugada syndrome: clinical, genetic, molecular, cellular, and ionic aspects. Curr Probl Cardiol. 2016;41(1):7–57.

    Article  Google Scholar 

  6. Bayés de Luna A, Brugada J, Baranchuk A, et al. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol. 2012;45(5):433–42.

    Article  Google Scholar 

  7. Nademanee K. Sudden unexplained death syndrome in Southeast Asia. Am J Cardiol. 1997;79(6A):10–1.

    Article  CAS  Google Scholar 

  8. Baranchuk A, Nguyen T, Ryu MH, et al. Brugada phenocopy: new terminology and proposed classification. Ann Noninvasive Electrocardiol. 2012;17(4):299–314.

    Article  Google Scholar 

  9. Dendramis G. Brugada syndrome and Brugada phenocopy. The importance of a differential diagnosis. Int J Cardiol. 2016;210:25–7.

    Article  Google Scholar 

  10. Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2015;36:2793–867.

    Article  Google Scholar 

  11. Adler A, Rosso R, Chorin E, et al. Risk stratification in Brugada syndrome: clinical characteristics, electrocardiographic parameters, and auxiliary testing. Heart Rhythm. 2016;13(1):299–310.

    Article  Google Scholar 

  12. Morita H, Kusano-Fukushima K, Nagase S, et al. Atrial fibrillation and atrial vulnerability in patients with Brugada syndrome. J Am Coll Cardiol. 2002;40(8):1437–44.

    Article  Google Scholar 

  13. Rodríguez-Mañero M, Namdar M, Sarkozy A, et al. Prevalence, clinical characteristics and management of atrial fibrillation in patients with Brugada syndrome. Am J Cardiol. 2013;111(3):362–7.

    Article  Google Scholar 

  14. Giustetto C, Cerrato N, Gribaudo E, et al. Atrial fibrillation in a large population with Brugada electrocardiographic pattern: prevalence, management, and correlation with prognosis. Heart Rhythm. 2014;11(2):259–65.

    Article  Google Scholar 

  15. Letsas KP, Korantzopoulos P, Efremidis M, et al. Sinus node disease in subjects with type 1 ECG pattern of Brugada syndrome. J Cardiol. 2013;61(3):227–31.

    Article  Google Scholar 

  16. Morita H. Identification of electrocardiographic risk markers for the initial and recurrent episodes of ventricular fibrillation in patients with Brugada syndrome. J Cardiovasc Electrophysiol. 2017;29(1):107–14.

    Article  Google Scholar 

  17. Antzelevitch C, Pollevick GD, Cordeiro JM, et al. Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST-segment elevation, short QT intervals, and sudden cardiac death. Circulation. 2007;115(4):442–9.

    Article  Google Scholar 

  18. Giustetto C, Schimpf R, Mazzanti A, et al. Long-term follow-up of patients with short QT syndrome. J Am Coll Cardiol. 2011;58(6):587–95.

    Article  Google Scholar 

  19. Gaita F, Giustetto C, Bianchi F, et al. Short QT syndrome: pharmacological treatment. J Am Coll Cardiol. 2004;43(8):1494–9.

    Article  CAS  Google Scholar 

  20. O'Rourke MF, Avolio AP, Nichols WW, et al. The kangaroo as a model for the study of hypertrophic cardiomyopathy in man. Cardiovasc Res. 1986;20(6):398–402.

    Article  CAS  Google Scholar 

  21. Algra A, Tijssen JG, Roelandt JR, et al. QT interval variables from 24 hour electrocardiography and the two year risk of sudden death. Br Heart J. 1993;70(1):43–8.

    Article  CAS  Google Scholar 

  22. Gussak I, Brugada P, Brugada J, et al. Idiopathic short QT interval: a new clinical syndrome? Cardiology. 2003;94(2):99–102.

    Article  Google Scholar 

  23. Gaita F, Giustetto C, Bianchi F, et al. Short QT Syndrome: a familial cause of sudden death. Circulation. 2003;108(8):965–70.

    Article  Google Scholar 

  24. Anttonen O, Junttila MJ, Rissanen H, et al. Prevalence and prognostic significance of short QT interval in a middle-aged Finnish population. Circulation. 2007;116(7):714–20.

    Article  CAS  Google Scholar 

  25. Mazzanti A, Kanthan A, Monteforte N, et al. Novel insight into the natural history of short QT syndrome. J Am Coll Cardiol. 2014;63(13):1300–8.

    Article  Google Scholar 

  26. Priori SG, Blomström-Lundqvist C, Mazzanti A, et al. ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015;36(41):2793–867.

    Article  Google Scholar 

  27. Giustetto C, Scrocco C, Schimpf R, et al. Usefulness of exercise test in the diagnosis of short QT syndrome. Europace. 2015;17(4):628–34.

    Article  Google Scholar 

  28. Postema PG, Wilde AA. The measurement of the QT interval. Curr Cardiol Rev. 2014;10(3):287–94.

    Article  Google Scholar 

  29. Moss AJ. Measurement of the QT interval and the risk associated with QTc interval prolongation: a review. Am J Cardiol. 1993;72(6):23B–5B.

    Article  CAS  Google Scholar 

  30. Lepeschkin E, Surawicz B. The measurement of the Q-T interval of the electrocardiogram. Circulation. 1952;6(3):378–88.

    Article  CAS  Google Scholar 

  31. Goldenberg I, Moss AJ, Zareba W. QT interval: how to measure it and what is “normal”. J Cardiovasc Electrophysiol. 2006;17(3):333–6.

    Article  Google Scholar 

  32. Bazett HC. An analysis of the time-relations of electrocardiograms. Heart. 1920;7:353–70.

    Google Scholar 

  33. Mazzanti A, Underwood K, Nevelev D, et al. The new kids on the block of arrhythmogenic disorders: short QT syndrome and early repolarization. J Cardiovasc Electrophysiol. 2017;28(10):1226–36.

    Article  Google Scholar 

  34. Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies. Europace. 2011;13:1077–109.

    Article  Google Scholar 

  35. Lu JT, Kass RS. Recent progress in congenital long QT syndrome. Curr Opin Cardiol. 2010;25:216–21.

    Article  Google Scholar 

  36. Sauer AJ, Moss AJ, McNitt S, et al. Long QT syndrome in adults. J Am Coll Cardiol. 2007;49:329–37.

    Article  Google Scholar 

  37. Goldenberg I, Moss AJ. Long QT syndrome. J Am Coll Cardiol. 2008;51:2291–300.

    Article  Google Scholar 

  38. Saenen JB, Vrints CJ. Molecular aspects of the congenital and acquired long QT syndrome: clinical implications. J Mol Cell Cardiol. 2008;44:633–46.

    Article  CAS  Google Scholar 

  39. Sy RW, Chattha IS, Klein GJ, et al. Repolarization dynamics during exercise discriminate between LQT1 and LQT2 genotypes. J Cardiovasc Electrophysiol. 2010;21:1242–6.

    Article  Google Scholar 

  40. Ruan Y, Liu N, Napolitano C, Priori SG. Therapeutic strategies for long-QT syndrome: does the molecular substrate matter? Circ Arrhythm Electrophysiol. 2008;1:290–7.

    Article  Google Scholar 

  41. Moss AJ, Goldenberg I. Importance of knowing the genotype and the specific mutation when managing patients with long QT syndrome. Circ Arrhythm Electrophysiol. 2008;1:213–26.

    Article  Google Scholar 

  42. Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation. 2001;103:89.

    Article  CAS  Google Scholar 

  43. Shimizu W, Noda T, Takaki H, et al. Diagnostic value of epinephrine test for genotyping LQT1, LQT2, and LQT3 forms of congenital long QT syndrome. Heart Rhythm. 2004;1:276–83.

    Article  Google Scholar 

  44. Vyas H, Hejilik J, Ackerman MJ. Epinephrine QT stress testing in the evaluation of congenital long QT syndrome: diagnostic accuracy of the paradoxical QT response. Circulation. 2006;113(11):1385–92.

    Article  CAS  Google Scholar 

  45. Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Europace. 2006;8:746–837.

    Article  Google Scholar 

  46. Rautaharju PM, Surawicz B, Gettes LS, et al. AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram. IV. The ST segment, T and U waves, and the QT interval: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society. Endorsed by the International Society for Computerized Electrocardiology. J Am Coll Cardiol. 2009;53(11):982–91.

    Article  Google Scholar 

  47. Chiladakis J, Kalogeropoulos A, Arvanitis P, et al. Preferred QT correction formula for the assessment of drug-induced QT interval prolongation. J Cardiovasc Electrophysiol. 2010;21:905–13.

    PubMed  Google Scholar 

  48. Monnig G, Eckardt L, Wedekind H, et al. Electrocardiographic risk stratification in families with congenital long QT syndrome. Eur Heart J. 2006;27:2074–80.

    Article  Google Scholar 

  49. Malik M, Hnatkova K, Schmidt A, Smetana P. Accurately measured and properly heart-rate corrected QTc intervals show little daytime variability. Heart Rhythm. 2008;5:1424–31.

    Article  Google Scholar 

  50. Viskin S. The QT interval: too long, too short or just right. Heart Rhythm. 2009;6:711–5.

    Article  Google Scholar 

  51. Goldenberg I, Mathew J, Moss AJ, et al. Corrected QT variability in serial electrocardiograms in long QT syndrome: the importance of the maximum corrected QT for risk stratification. J Am Coll Cardiol. 2006;48:1047–52.

    Article  Google Scholar 

  52. Viitasalo M, Oikarinen L, Swan H, et al. Ambulatory electrocardiographic evidence of transmural dispersion of repolarization in patients with long-QT syndrome type 1 and 2. Circulation. 2002;106:2473–8.

    Article  Google Scholar 

  53. Antzelevitch C, Shimizu W, Yan GX, Sicouri S, Weissenburger J, Nesterenko VV, et al. The M cell: its contribution to the ECG and to normal and abnormal electrical function of the heart. J Cardiovasc Electrophysiol. 1999;10(8):1124–52.

    Article  CAS  Google Scholar 

  54. Rautaharju PM, Surawicz B, Gettes LS, et al. AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram. IV. The ST segment, T and U waves, and the QT interval: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society. J Am Coll Cardiol. 2009;53:982–91.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Clinica di Cardiologia e Aritmologia, Ospedali Riuniti di Ancona, Ancona, Italy

    Paolo Compagnucci, Simone D’Agostino, Alessia Quaranta & Giulio Spinucci

Authors
  1. Paolo Compagnucci
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Simone D’Agostino
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Alessia Quaranta
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Giulio Spinucci
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Cardiology Clinic, Marche Polytechnic University Cardiology Clinic, Ancona, Italy

    Alessandro Capucci

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Compagnucci, P., D’Agostino, S., Quaranta, A., Spinucci, G. (2019). Channellopathies: New ECG Criteria for Risk Stratification. In: Capucci, A. (eds) New Concepts in ECG Interpretation. Springer, Cham. https://doi.org/10.1007/978-3-319-91677-4_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-91677-4_12

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-91676-7

  • Online ISBN: 978-3-319-91677-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation