Skip to main content
SpringerLink
Log in

J Wave Syndromes: From Bench to Bedside

  • Chapter
  • First Online:
J Wave Syndromes

  • 674 Accesses

Abstract

J wave syndromes represent a spectrum of electrocardiographic manifestations of ventricular repolarization abnormalities characterized by prominent J waves and the potential to predispose affected individuals to polymorphic ventricular tachycardia (VT) or ventricular fibrillation (VF). J wave syndromes include two major forms: early repolarization syndrome and Brugada syndrome. These syndromes share a common ionic and cellular mechanism with prominent Ito-mediated action potential (AP) spike and dome in ventricular epicardium and are linked by J wave on the ECG. When Ito is large, an increase in net repolarization current by either a decrease in an inward current (INa or ICa) or an increase in an outward potassium current (Ito per se or IK-ATP) predisposes loss of epicardial AP dome. Loss of epicardial AP dome is often heterogeneous, resulting in two important changes at cellular levels that facilitates the development of polymorphic VT/VF: (1) the epicardial AP dome in some sites may propagate to sites where the cells has completely lost their dome, leading to phase 2 reentry that manifests short-coupled extrasystoles on the ECG; (2) an increase in transmural dispersion of repolarization that can serve as reentry substrate and manifest as downslope ST segment elevation. This chapter attempts to translate the abnormalities at cellular level associated with J wave syndromes to their clinical ECG manifestations.

This study was supported by the Sharpe-Strumia Research Foundation and National Natural Science Foundation of China (NSFC-81370289).

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 179.99
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

References

  1. Yan GX, Antzelevitch C. Cellular basis for the electrocardiographic J wave. Circulation. 1996;93:372–9.

    Article  CAS  PubMed  Google Scholar 

  2. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. 1992;20:1391–6.

    Article  CAS  PubMed  Google Scholar 

  3. Kalla H, Yan GX, Marinchak R. Ventricular fibrillation in a patient with prominent J (Osborn) waves and ST segment elevation in the inferior electrocardiographic leads: a Brugada syndrome variant? J Cardiovasc Electrophysiol. 2000;11:95–8.

    Article  CAS  PubMed  Google Scholar 

  4. Takagi M, Aihara N, Takaki H, Taguchi A, Shimizu W, Kurita T, Suyama K, Kamakura S. Clinical characteristics of patients with spontaneous or inducible ventricular fibrillation without apparent heart disease presenting with J wave and ST segment elevation in inferior leads. J Cardiovasc Electrophysiol. 2000;11:844–8.

    Article  CAS  PubMed  Google Scholar 

  5. Qi X, Sun F, An X, Yang J. A case of Brugada syndrome with ST segment elevation through entire precordial leads. Chin J Cardiol. 2004;32:272–3.

    Google Scholar 

  6. Yan GX, Yao QH, Wang DQ, Cui CC. Electrocardiographic J wave and J wave syndromes. Chin J Card Arrhyth. 2004;8:360–5.

    Google Scholar 

  7. Shu J, Zhu T, Yang L, Cui C, Yan GX. ST-segment elevation in the early repolarization syndrome, idiopathic ventricular fibrillation, and the Brugada syndrome: cellular and clinical linkage. J Electrocardiol. 2005;38:26–32.

    Article  PubMed  Google Scholar 

  8. Hlaing T, DiMino T, Kowey PR, Yan GX. ECG repolarization waves: their genesis and clinical implications. Ann Noninvasive Electrocardiol. 2005;10:211–23.

    Article  PubMed  Google Scholar 

  9. Haissaguerre M, Derval N, Sacher F, Jesel L, Deisenhofer I, de Roy L, Pasquie JL, Nogami A, Babuty D, Yli-Mayry S, De CC, Scanu P, Mabo P, Matsuo S, Probst V, Le SS, Defaye P, Schlaepfer J, Rostock T, Lacroix D, Lamaison D, Lavergne T, Aizawa Y, Englund A, Anselme F, O’Neill M, Hocini M, Lim KT, Knecht S, Veenhuyzen GD, Bordachar P, Chauvin M, Jais P, Coureau G, Chene G, Klein GJ, Clementy J. Sudden cardiac arrest associated with early repolarization. N Engl J Med. 2008;358:2016–23.

    Article  CAS  PubMed  Google Scholar 

  10. Nam GB, Kim YH, Antzelevitch C. Augmentation of J waves and electrical storms in patients with early repolarization. N Engl J Med. 2008;358:2078–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Antzelevitch C, Yan GX. J wave syndromes. Heart Rhythm. 2010;7:549–58.

    Article  PubMed  Google Scholar 

  12. Antzelevitch C, Yan GX. J wave syndromes: Brugada and early repolarization syndromes. Heart Rhythm. 2015;12:1852–66.

    Article  PubMed  Google Scholar 

  13. Litovsky SH, Antzelevitch C. Transient outward current prominent in canine ventricular epicardium but not endocardium. Circ Res. 1988;62:116–26.

    Article  CAS  PubMed  Google Scholar 

  14. Wasserburger RH, Alt WJ. The normal RS-T segment elevation variant. Am J Cardiol. 1961;8:184–92.

    Article  CAS  PubMed  Google Scholar 

  15. Yan GX, Lankipalli RS, Burke JF, Musco S, Kowey PR. Ventricular repolarization components on the electrocardiogram: cellular basis and clinical significance. J Am Coll Cardiol. 2003;42:401–9.

    Article  PubMed  Google Scholar 

  16. Tikkanen JT, Anttonen O, Junttila MJ, Aro AL, Kerola T, Rissanen HA, Reunanen A, Huikuri HV. Long-term outcome associated with early repolarization on electrocardiography. N Engl J Med. 2009;361:2529–37.

    Article  CAS  PubMed  Google Scholar 

  17. Tikkanen JT, Junttila MJ, Anttonen O, Aro AL, Luttinen S, Kerola T, Sager SJ, Rissanen HA, Myerburg RJ, Reunanen A, Huikuri HV. Early repolarization: electrocardiographic phenotypes associated with favorable long-term outcome. Circulation. 2011;123:2666–73.

    Article  PubMed  Google Scholar 

  18. Rosso R, Glikson E, Belhassen B, Katz A, Halkin A, Steinvil A, Viskin S. Distinguishing “benign” from “malignant early repolarization”: the value of the ST-segment morphology. Heart Rhythm. 2012;9:225–9.

    Article  PubMed  Google Scholar 

  19. Rollin A, Maury P, Bongard V, Sacher F, Delay M, Duparc A, Mondoly P, Carrie D, Ferrieres J, Ruidavets JB. Prevalence, prognosis, and identification of the malignant form of early repolarization pattern in a population-based study. Am J Cardiol. 2012;110:1302–8.

    Article  PubMed  Google Scholar 

  20. Badri M, Patel A, Yan GX. Cellular and ionic basis of J-wave syndromes. Trends Cardiovasc Med. 2015;25:12–21.

    Article  CAS  PubMed  Google Scholar 

  21. Yan GX, Antzelevitch C. Cellular basis for the Brugada syndrome and other mechanisms of arrhythmogenesis associated with ST-segment elevation. Circulation. 1999;100:1660–6.

    Article  CAS  PubMed  Google Scholar 

  22. Aizawa Y, Sato A, Watanabe H, Chinushi M, Furushima H, Horie M, Kaneko Y, Imaizumi T, Okubo K, Watanabe I, Shinozaki T, Aizawa Y, Fukuda K, Joo K, Haissaguerre M. Dynamicity of the J-wave in idiopathic ventricular fibrillation with a special reference to pause-dependent augmentation of the J-wave. J Am Coll Cardiol. 2012;59:1948–53.

    Article  PubMed  Google Scholar 

  23. Nademanee K, Veerakul G, Nimmannit S, Chaowakul V, Bhuripanyo K, Likittanasombat K, Tunsanga K, Kuasirikul S, Malasit P, Tansupasawadikul S, Tatsanavivat P. Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men. Circulation. 1997;96:2595–600.

    Article  CAS  PubMed  Google Scholar 

  24. Aizawa Y, Tamura M, Chinushi M, Naitoh N, Uchiyama H, Kusano Y, Hosono H, Shibata A. Idiopathic ventricular fibrillation and bradycardia-dependent intraventricular block. Am Heart J. 1993;126:1473–4.

    Article  CAS  PubMed  Google Scholar 

  25. Yan GX, Wu Y, Liu T, Wang J, Marinchak RA, Kowey PR. Phase 2 early afterdepolarization as a trigger of polymorphic ventricular tachycardia in acquired long-QT syndrome: direct evidence from intracellular recordings in the intact left ventricular wall. Circulation. 2001;103:2851–6.

    Article  CAS  PubMed  Google Scholar 

  26. Bloch Thomsen PE, Joergensen RM, Kanters JK, Jensen TJ, Haarbo J, Hagemann A, Vestergaard A, Saermark K. Phase 2 reentry in man. Heart Rhythm. 2005;2:797–803.

    Article  PubMed  Google Scholar 

  27. Szel T, Antzelevitch C. Abnormal repolarization as the basis for late potentials and fractionated electrograms recorded from epicardium in experimental models of Brugada syndrome. J Am Coll Cardiol. 2014;63:2037–45.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Nademanee K, Veerakul G, Chandanamattha P, Chaothawee L, Ariyachaipanich A, Jirasirirojanakorn K, Likittanasombat K, Bhuripanyo K, Ngarmukos T. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation. 2011;123:1270–9.

    Article  PubMed  Google Scholar 

  29. Sacher F, Derval N, Horlitz M, Haissaguerre M. J wave elevation to monitor quinidine efficacy in early repolarization syndrome. J Electrocardiol. 2014;47:223–5.

    Article  PubMed  Google Scholar 

  30. Benito B, Sarkozy A, Mont L, Henkens S, Berruezo A, Tamborero D, Arzamendi D, Berne P, Brugada R, Brugada P, Brugada J. Gender differences in clinical manifestations of Brugada syndrome. J Am Coll Cardiol. 2008;52:1567–73.

    Article  PubMed  Google Scholar 

  31. Di Diego JM, Cordeiro JM, Goodrow RJ, Fish JM, Zygmunt AC, Perez GJ, Scornik FS, Antzelevitch C. Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males. Circulation. 2002;106:2004–11.

    Article  PubMed  Google Scholar 

  32. Shimizu W, Matsuo K, Kokubo Y, Satomi K, Kurita T, Noda T, Nagaya N, Suyama K, Aihara N, Kamakura S, Inamoto N, Akahoshi M, Tomoike H. Sex hormone and gender difference – role of testosterone on male predominance in Brugada syndrome. J Cardiovasc Electrophysiol. 2007;18:415–21.

    Article  PubMed  Google Scholar 

  33. Matsuo K, Akahoshi M, Seto S, Yano K. Disappearance of the Brugada-type electrocardiogram after surgical castration: a role for testosterone and an explanation for the male preponderance. Pacing Clin Electrophysiol. 2003;26:1551–3.

    Article  PubMed  Google Scholar 

  34. US Centers for Disease Control. Sudden, unexpected, nocturnal deaths among Southeast Asian refugees. MMWR Morb Mortal Wkly Rep. 1981;30:581–4, 589.

    Google Scholar 

  35. Otto CM, Tauxe RV, Cobb LA, Greene HL, Gross BW, Werner JA, Burroughs RW, Samson WE, Weaver WD, Trobaugh GB. Ventricular fibrillation causes sudden death in Southeast Asian immigrants. Ann Intern Med. 1984;100:45–7.

    Article  Google Scholar 

  36. Matsuo K, Kurita T, Inagaki M, Kakishita M, Aihara N, Shimizu W, Taguchi A, Suyama K, Kamakura S, Shimomura K. The circadian pattern of the development of ventricular fibrillation in patients with Brugada syndrome. Eur Heart J. 1999;20:465–70.

    Article  CAS  PubMed  Google Scholar 

  37. Mizumaki K, Nishida K, Iwamoto J, Nakatani Y, Yamaguchi Y, Sakamoto T, Tsuneda T, Kataoka N, Inoue H. Vagal activity modulates spontaneous augmentation of J-wave elevation in patients with idiopathic ventricular fibrillation. Heart Rhythm. 2012;9:249–55.

    Article  PubMed  Google Scholar 

  38. Ikeda T, Abe A, Yusu S, Nakamura K, Ishiguro H, Mera H, Yotsukura M, Yoshino H. The full stomach test as a novel diagnostic technique for identifying patients at risk of Brugada syndrome. J Cardiovasc Electrophysiol. 2006;17:602–7.

    Article  PubMed  Google Scholar 

  39. Sealy WC, Johnson PA, Lesage AM, Young Jr WG. Effect of quinidine on ventricular fibrillation in hypothermic dogs. Proc Soc Exp Biol Med. 1959;101:456–7.

    Article  CAS  PubMed  Google Scholar 

  40. Johnson P, Lesage A, Floyd WL, Young Jr WG, Sealy WC. Prevention of ventricular fibrillation during profound hypothermia by quinidine. Ann Surg. 1960;151:490–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Yan GX, Joshi A, Guo D, Hlaing T, Martin J, Xu X, Kowey PR. Phase 2 reentry as a trigger to initiate ventricular fibrillation during early acute myocardial ischemia. Circulation. 2004;110:1036–41.

    Article  PubMed  Google Scholar 

  42. Link MS, Wang PJ, Maron BJ, Estes NA. What is commotio cordis? Cardiol Rev. 1999;7:265–9.

    Article  CAS  PubMed  Google Scholar 

  43. Baranchuk A, Barbosa-Barros R, Perez-Riera AR. Brugada ECG pattern obscured by right bundle branch block: how to resolve the enigma? Pacing Clin Electrophysiol. 2014;37:1071–2.

    Article  PubMed  Google Scholar 

  44. Shiue HC, Divakaran VG, Lakkis NM. A case of Brugada syndrome presenting with incessant polymorphic ventricular tachycardia. Clin Cardiol. 2010;33:E33–5.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Cardiology, Lankenau Medical Center, Lankenau Institute for Medical Research, 100 Lancaster Ave., LIMR Suite 230, Wynnewood, PA, 19096, USA

    Gan-Xin Yan MD, PhD.

  2. Cardiology, Anzhen Hospital, Capital Medical University, Beijing, China

    Gan-Xin Yan MD, PhD. & Jian-Zeng Dong MD, PhD

  3. Cardiology, The First Affiliated Hospital of Xi’an Jiaotong University, Xi’An, Shanxi, China

    Chang-Cong Cui MD

Authors
  1. Gan-Xin Yan MD, PhD.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jian-Zeng Dong MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Chang-Cong Cui MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gan-Xin Yan MD, PhD. .

Editor information

Editors and Affiliations

  1. Cardiovascular Research, Lankenau Institute for Medical Research, Wynnewood, Pennsylvania, USA

    Charles Antzelevitch

  2. Lankenau Institute for Medical Research, Lankenau Medical Center, Wynnewood, Pennsylvania, USA

    Gan-Xin Yan

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Yan, GX., Dong, JZ., Cui, CC. (2016). J Wave Syndromes: From Bench to Bedside. In: Antzelevitch, C., Yan, GX. (eds) J Wave Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-31578-2_2

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-31578-2_2

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-31576-8

  • Online ISBN: 978-3-319-31578-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation