Skip to main content

Clinical Features of Electrical Storms Associated with J Wave Syndromes and Acquired Forms of J Wave Syndrome

  • Chapter
  • First Online:
  • 665 Accesses

Abstract

Recurrent, life-threatening ventricular tachyarrhythmias (VTAs) or electrical storm (ES) have been well described in patients with structural heart diseases. However, there are only limited data on ES in patients with J wave syndrome (JWS). ES is an important clinical manifestation of JWS. It can be the first manifestation of JWS, and if not properly managed, may lead to catastrophic outcomes.

When monitored vigilantly, ES is accompanied by dramatic ECG changes. Ventricular fibrillation (VF) episodes are typically initiated by bradycardia or sinus pauses. Prominent bradycardia-dependent augmentation of ER patterns or right precordial J waves is noted before initiation of VF. Traditional antiarrhythmic medications are largely not effective. However, they show dramatic response to intravenous isoproterenol or heart rate elevation. For long-term management of VF episodes, oral quinidine and implantable defibrillators are indicated.

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

Buying options

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

Learn about institutional subscriptions

References

  1. Kowey PR, Levine JH, Herre JM, Pacifico A, Lindsay BD, Plumb VJ, Janosik DL, Kopelman HA, Scheinman MM. Randomized, double-blind comparison of intravenous amiodarone and bretylium in the treatment of patients with recurrent, hemodynamically destabilizing ventricular tachycardia or fibrillation. The intravenous amiodarone multicenter investigators group. Circulation. 1995;92:3255–63.

    Article  CAS  PubMed  Google Scholar 

  2. Credner SC, Klingenheben T, Mauss O, Sticherling C, Hohnloser SH. Electrical storm in patients with transvenous implantable cardioverter-defibrillators: incidence, management and prognostic implications. J Am Coll Cardiol. 1998;32:1909–15.

    Article  CAS  PubMed  Google Scholar 

  3. Chalvidan T, Deharo JC, Dieuzaide P, Defaye P, Djiane P. Near fatal electrical storm in a patient equipped with an implantable cardioverter defibrillator for brugada syndrome. Pacing Clin Electrophysiol PACE. 2000;23:410–2.

    Article  CAS  PubMed  Google Scholar 

  4. Pagel PS, Lilly RE, Nicolosi AC. Use of ecmo to temporize circulatory instability during severe brugada electrical storm. Ann Thorac Surg. 2009;88:982–3.

    Article  PubMed  Google Scholar 

  5. Pallisgaard JL, Gang U, Kanters JK, Hansen PR. Massive electrical storm at disease onset in a patient with brugada syndrome. Am J Case Rep. 2014;15:559–61.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Ayerza MR, de Zutter M, Goethals M, Wellens F, Geelen P, Brugada P. Heart transplantation as last resort against brugada syndrome. J Cardiovasc Electrophysiol. 2002;13:943–4.

    Article  PubMed  Google Scholar 

  7. Suzuki H, Torigoe K, Numata O, Yazaki S. Infant case with a malignant form of brugada syndrome. J Cardiovasc Electrophysiol. 2000;11:1277–80.

    Article  CAS  PubMed  Google Scholar 

  8. Maury P, Couderc P, Delay M, Boveda S, Brugada J. Electrical storm in brugada syndrome successfully treated using isoprenaline. Europace Eur Pacing, Arrhythmias, Card Electrophysiol J Work Groups Card Pacing, Arrhythmias Card Cell Electrophysiol Eur Soc Cardiol. 2004;6:130–3.

    Article  CAS  Google Scholar 

  9. Nakamura M, Isobe M, Imamura H. Incessant ventricular fibrillation attacks in a patient with brugada syndrome. Int J Cardiol. 1998;64:205–6.

    Article  CAS  PubMed  Google Scholar 

  10. Watanabe H, Chinushi M, Washizuka T, Sugiura H, Hirono T, Komura S, Hosaka Y, Yamaura M, Tanabe Y, Furushima H, Fujita S, Aizawa Y. Variable electrocardiographic effects of short-term quinidine sulfate administration in brugada syndrome. Pacing Clin Electrophysiol PACE. 2005;28:372–7.

    Article  PubMed  Google Scholar 

  11. Haghjoo M, Arya A, Heidari A, Sadr-Ameli MA. Suppression of electrical storm by oral quinidine in a patient with brugada syndrome. J Cardiovasc Electrophysiol. 2005;16:674.

    Article  PubMed  Google Scholar 

  12. Jongman JK, Jepkes-Bruin N, Ramdat Misier AR, Beukema WP, Delnoy PP, Oude Lutttikhuis H, Dambrink JH, Hoorntje JC, Elvan A. Electrical storms in brugada syndrome successfully treated with isoproterenol infusion and quinidine orally. Neth Heart J Mon J Neth Soc Cardiol Neth Heart Found. 2007;15:151–5.

    Article  CAS  Google Scholar 

  13. Schweizer PA, Becker R, Katus HA, Thomas D. Successful acute and long-term management of electrical storm in brugada syndrome using orciprenaline and quinine/quinidine. Clin Res Cardiol Off Journal German Card Soc. 2010;99:467–70.

    Article  Google Scholar 

  14. Kyriazis K, Bahlmann E, van der Schalk H, Kuck KH. Electrical storm in brugada syndrome successfully treated with orciprenaline; effect of low-dose quinidine on the electrocardiogram. Europace Eur Pacing, Arrhythmias, Card Electrophysiol J Working Groups on Card Pacing, Arrhythmias, Card Cell Electrophysiol Eur Soc Cardiol. 2009;11:665–6.

    Article  Google Scholar 

  15. Bernard A, Genee O, Grimard C, Sacher F, Fauchier L, Babuty D. Electrical storm reversible by isoproterenol infusion in a striking case of early repolarization. J Interv Card Electrophysiol Int J Arrhythmias Pacing. 2009;25:123–7.

    Article  Google Scholar 

  16. Myojo T, Sato N, Nimura A, Matsuo A, Taniguchi O, Nakamura H, Karim Talib A, Sakamoto N, Takeuchi T, Kawamura Y, Hasebe N. Recurrent ventricular fibrillation related to hypokalemia in early repolarization syndrome. Pacing Clin Electrophysiol PACE. 2012;35:e234–8.

    Article  PubMed  Google Scholar 

  17. Ohgo T, Okamura H, Noda T, Satomi K, Suyama K, Kurita T, Aihara N, Kamakura S, Ohe T, Shimizu W. Acute and chronic management in patients with brugada syndrome associated with electrical storm of ventricular fibrillation. Heart Rhythm Off J Heart Rhythm Soc. 2007;4:695–700.

    Article  Google Scholar 

  18. Kaneko Y, Horie M, Niwano S, Kusano KF, Takatsuki S, Kurita T, Mitsuhashi T, Nakajima T, Irie T, Hasegawa K, Noda T, Kamakura S, Aizawa Y, Yasuoka R, Torigoe K, Suzuki H, Ohe T, Shimizu A, Fukuda K, Kurabayashi M, Aizawa Y. Electrical storm in patients with brugada syndrome is associated with early repolarization. Circ Arrhythm Electrophysiol. 2014;7:1122–8.

    Article  PubMed  Google Scholar 

  19. Conte G, Sieira J, Ciconte G, de Asmundis C, Chierchia GB, Baltogiannis G, Di Giovanni G, La Meir M, Wellens F, Czapla J, Wauters K, Levinstein M, Saitoh Y, Irfan G, Julia J, Pappaert G, Brugada P. Implantable cardioverter-defibrillator therapy in brugada syndrome: a 20-year single-center experience. J Am Coll Cardiol. 2015;65:879–88.

    Article  PubMed  Google Scholar 

  20. Haissaguerre M, Sacher F, Nogami A, Komiya N, Bernard A, Probst V, Yli-Mayry S, Defaye P, Aizawa Y, Frank R, Mantovan R, Cappato R, Wolpert C, Leenhardt A, de Roy L, Heidbuchel H, Deisenhofer I, Arentz T, Pasquie JL, Weerasooriya R, Hocini M, Jais P, Derval N, Bordachar P, Clementy J. Characteristics of recurrent ventricular fibrillation associated with inferolateral early repolarization role of drug therapy. J Am Coll Cardiol. 2009;53:612–9.

    Article  PubMed  Google Scholar 

  21. Nam GB, Ko KH, Kim J, Park KM, Rhee KS, Choi KJ, Kim YH, Antzelevitch C. Mode of onset of ventricular fibrillation in patients with early repolarization pattern vs. Brugada syndrome. Eur Heart J. 2010;31:330–9.

    Article  PubMed  Google Scholar 

  22. Aizawa Y, Chinushi M, Hasegawa K, Naiki N, Horie M, Kaneko Y, Kurabayashi M, Ito S, Imaizumi T, Aizawa Y, Takatsuki S, Joo K, Sato M, Ebe K, Hosaka Y, Haissaguerre M, Fukuda K. Electrical storm in idiopathic ventricular fibrillation is associated with early repolarization. J Am Coll Cardiol. 2013;62:1015–9.

    Article  PubMed  Google Scholar 

  23. Sarkozy A, Chierchia GB, Paparella G, Boussy T, De Asmundis C, Roos M, Henkens S, Kaufman L, Buyl R, Brugada R, Brugada J, Brugada P. Inferior and lateral electrocardiographic repolarization abnormalities in brugada syndrome. Circ Arrhythm Electrophysiol. 2009;2:154–61.

    Article  PubMed  Google Scholar 

  24. Takagi M, Aonuma K, Sekiguchi Y, Yokoyama Y, Aihara N, Hiraoka M. The prognostic value of early repolarization (j wave) and st-segment morphology after j wave in brugada syndrome: Multicenter study in japan. Heart Rhythm Off J Heart Rhythm Soc. 2013;10:533–9.

    Article  Google Scholar 

  25. Kawata H, Morita H, Yamada Y, Noda T, Satomi K, Aiba T, Isobe M, Nagase S, Nakamura K, Fukushima Kusano K, Ito H, Kamakura S, Shimizu W. Prognostic significance of early repolarization in inferolateral leads in brugada patients with documented ventricular fibrillation: A novel risk factor for brugada syndrome with ventricular fibrillation. Heart Rhythm Official J Heart Rhythm Soc. 2013;10:1161–8.

    Article  Google Scholar 

  26. Mok NS, Chan NY, Chiu AC. Successful use of quinidine in treatment of electrical storm in brugada syndrome. Pacing Clin Electrophysiol PACE. 2004;27:821–3.

    Article  PubMed  Google Scholar 

  27. Yang DH, Kwon HJ, Kim JC, Jang JH, Shin SH, Kwan J, Woo SI, Park KS, Kim DH. Transient j-wave appearance in the inferior-lateral leads during electrical storm in a patient with brugada syndrome. Korean Circ J. 2013;43:193–5.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Peters S. Early repolarization pattern in patients with provocable brugada phenocopy: a marker of additional arrhythmogenic cardiomyopathy? Int J Cardiol. 2013;168:4928–9.

    Article  PubMed  Google Scholar 

  29. McIntyre WF, Perez-Riera AR, Femenia F, Baranchuk A. Coexisting early repolarization pattern and brugada syndrome: recognition of potentially overlapping entities. J Electrocardiol. 2012;45:195–8.

    Article  PubMed  Google Scholar 

  30. Kamakura T, Kawata H, Nakajima I, Yamada Y, Miyamoto K, Okamura H, Noda T, Satomi K, Aiba T, Takaki H, Aihara N, Kamakura S, Kimura T, Shimizu W. Significance of non-type 1 anterior early repolarization in patients with inferolateral early repolarization syndrome. J Am Coll Cardiol. 2013;62:1610–8.

    Article  PubMed  Google Scholar 

  31. Kasanuki H, Ohnishi S, Ohtuka M, Matsuda N, Nirei T, Isogai R, Shoda M, Toyoshima Y, Hosoda S. Idiopathic ventricular fibrillation induced with vagal activity in patients without obvious heart disease. Circulation. 1997;95:2277–85.

    Article  CAS  PubMed  Google Scholar 

  32. Kakishita M, Kurita T, Matsuo K, Taguchi A, Suyama K, Shimizu W, Aihara N, Kamakura S, Yamamoto F, Kobayashi J, Kosakai Y, Ohe T. Mode of onset of ventricular fibrillation in patients with brugada syndrome detected by implantable cardioverter defibrillator therapy. J Am Coll Cardiol. 2000;36:1646–53.

    Article  CAS  PubMed  Google Scholar 

  33. Haissaguerre M, Extramiana F, Hocini M, Cauchemez B, Jais P, Cabrera JA, Farre J, Leenhardt A, Sanders P, Scavee C, Hsu LF, Weerasooriya R, Shah DC, Frank R, Maury P, Delay M, Garrigue S, Clementy J. Mapping and ablation of ventricular fibrillation associated with long-qt and brugada syndromes. Circulation. 2003;108:925–8.

    Article  PubMed  Google Scholar 

  34. Matsuo K, Shimizu W, Kurita T, Inagaki M, Aihara N, Kamakura S. Dynamic changes of 12-lead electrocardiograms in a patient with brugada syndrome. J Cardiovasc Electrophysiol. 1998;9:508–12.

    Article  CAS  PubMed  Google Scholar 

  35. 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 

  36. Belhassen B, Glick A, Viskin S. Efficacy of quinidine in high-risk patients with brugada syndrome. Circulation. 2004;110:1731–7.

    Article  PubMed  Google Scholar 

  37. Hermida JS, Denjoy I, Clerc J, Extramiana F, Jarry G, Milliez P, Guicheney P, Di Fusco S, Rey JL, Cauchemez B, Leenhardt A. Hydroquinidine therapy in brugada syndrome. J Am Coll Cardiol. 2004;43:1853–60.

    Article  CAS  PubMed  Google Scholar 

  38. Tsuchiya T, Ashikaga K, Honda T, Arita M. Prevention of ventricular fibrillation by cilostazol, an oral phosphodiesterase inhibitor, in a patient with brugada syndrome. J Cardiovasc Electrophysiol. 2002;13:698–701.

    Article  PubMed  Google Scholar 

  39. Shinohara T, Ebata Y, Ayabe R, Fukui A, Okada N, Yufu K, Nakagawa M, Takahashi N. Combination therapy of cilostazol and bepridil suppresses recurrent ventricular fibrillation related to j-wave syndromes. Heart Rhythm Off J Heart Rhythm Soc. 2014;11:1441–5.

    Article  Google Scholar 

  40. Choi HO, Nam GB, Jin ES, Kim KH, Kim SH, Hwang ES, Park KM, Kim J, Rhee KS, Choi KJ, Kim YH. Temporal variation and morphologic characteristics of j-waves in patients with early repolarisation syndrome. Heart. 2013;99:1818–24.

    Article  PubMed  Google Scholar 

  41. Kim SH, Nam GB, Baek S, Choi HO, Kim KH, Choi KJ, Joung B, Pak HN, Lee MH, Kim SS, Park SJ, On YK, Kim JS, Oh IY, Choi EK, Oh S, Choi YS, Choi JI, Park SW, Kim YH, Lee MY, Lim HE, Lee YS, Cho Y, Kim J, Lee DI, Cho DK, Kim YH. Circadian and seasonal variations of ventricular tachyarrhythmias in patients with early repolarization syndrome and brugada syndrome: analysis of patients with implantable cardioverter defibrillator. J Cardiovasc Electrophysiol. 2012;23:757–63.

    Article  PubMed  Google Scholar 

  42. Dinckal MH, Davutoglu V, Akdemir I, Soydinc S, Kirilmaz A, Aksoy M. Incessant monomorphic ventricular tachycardia during febrile illness in a patient with brugada syndrome: fatal electrical storm. Europace Eur Pacing, Arrhythmias, Card Electrophysiol J Work Groups Card Pacing, Arrhythmias, Card Cell Electrophysiol Eur Soc Cardiol. 2003;5:257–61.

    Article  CAS  Google Scholar 

  43. Iguchi K, Noda T, Kamakura S, Shimizu W. Beneficial effects of cilostazol in a patient with recurrent ventricular fibrillation associated with early repolarization syndrome. Heart Rhythm Off J Heart Rhythm Soc. 2013;10:604–6.

    Article  Google Scholar 

  44. Fish JM, Antzelevitch C. Link between hypothermia and the brugada syndrome. J Cardiovasc Electrophysiol. 2004;15:942–4.

    Article  PubMed  PubMed Central  Google Scholar 

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

    Article  Google Scholar 

  46. Indik JH, Ott P, Butman SM. Syncope with st-segment abnormalities resembling brugada syndrome due to reversible myocardial ischemia. Pacing Clin Electrophysiol PACE. 2002;25:1270–3.

    Article  PubMed  Google Scholar 

  47. Kataoka H. Electrocardiographic patterns of the brugada syndrome in right ventricular infarction/ischemia. Am J Cardiol. 2000;86:1056.

    Article  CAS  PubMed  Google Scholar 

  48. Kataoka H, Kanzaki K, Mikuriya Y. Massive st-segment elevation in precordial and inferior leads in right ventricular myocardial infarction. J Electrocardiol. 1988;21:115–20.

    Article  CAS  PubMed  Google Scholar 

  49. Yan GX, Kowey PR. St segment elevation and sudden cardiac death: From the brugada syndrome to acute myocardial ischemia. J Cardiovasc Electrophysiol. 2000;11:1330–2.

    Article  CAS  PubMed  Google Scholar 

  50. Gheeraert PJ, Henriques JP, De Buyzere ML, Voet J, Calle P, Taeymans Y, Zijlstra F. Out-of-hospital ventricular fibrillation in patients with acute myocardial infarction: coronary angiographic determinants. J Am Coll Cardiol. 2000;35:144–50.

    Article  CAS  PubMed  Google Scholar 

  51. Volpi A, Maggioni A, Franzosi MG, Pampallona S, Mauri F, Tognoni G. In-hospital prognosis of patients with acute myocardial infarction complicated by primary ventricular fibrillation. N Engl J Med. 1987;317:257–61.

    Article  CAS  PubMed  Google Scholar 

  52. Berger PB, Ruocco NA, Ryan TJ, Frederick MM, Podrid PJ. Incidence and significance of ventricular tachycardia and fibrillation in the absence of hypotension or heart failure in acute myocardial infarction treated with recombinant tissue-type plasminogen activator: results from the thrombolysis in myocardial infarction (timi) phase ii trial. J Am Coll Cardiol. 1993;22:1773–9.

    Article  CAS  PubMed  Google Scholar 

  53. Mehta SR, Eikelboom JW, Natarajan MK, Diaz R, Yi C, Gibbons RJ, Yusuf S. Impact of right ventricular involvement on mortality and morbidity in patients with inferior myocardial infarction. J Am Coll Cardiol. 2001;37:37–43.

    Article  CAS  PubMed  Google Scholar 

  54. Lerner DJ, Kannel WB. Patterns of coronary heart disease morbidity and mortality in the sexes: a 26-year follow-up of the Framingham population. Am Heart J. 1986;111:383–90.

    Article  CAS  PubMed  Google Scholar 

  55. Hu D, Viskin S, Oliva A, Carrier T, Cordeiro JM, Barajas-Martinez H, Wu Y, Burashnikov E, Sicouri S, Brugada R, Rosso R, Guerchicoff A, Pollevick GD, Antzelevitch C. Novel mutation in the scn5a gene associated with arrhythmic storm development during acute myocardial infarction. Heart Rhythm Off J Heart Rhythm Soc. 2007;4:1072–80.

    Article  Google Scholar 

  56. Noda T, Shimizu W, Taguchi A, Satomi K, Suyama K, Kurita T, Aihara N, Kamakura S. St-segment elevation and ventricular fibrillation without coronary spasm by intracoronary injection of acetylcholine and/or ergonovine maleate in patients with brugada syndrome. J Am Coll Cardiol. 2002;40:1841–7.

    Article  PubMed  Google Scholar 

  57. Lee KH, Park HW, Cho JG, Yoon NS, Kim SS, Rhew SH, Jeong YW, Jang SY, Cho JY, Jeong HC, Park KH, Sim DS, Yoon HJ, Kim KH, Hong YJ, Kim JH, Ahn Y, Jeong MH, Park JC. Predictors of recurrent sudden cardiac death in patients associated with coronary vasospasm. Int J Cardiol. 2014;172:460–1.

    Article  PubMed  Google Scholar 

  58. Pavlidis AN, Giannakopoulos A, Manolis AJ. Iatrogenic giant osborn waves. Circulation. 2010;122:1519.

    Article  PubMed  Google Scholar 

  59. Takahiro T, Kou S, Toshinobu Y, Yuichi H. Accidental hypothermia-induced electrical storm successfully treated with isoproterenol. Heart Rhythm Off J Heart Rhythm Soc. 2015;12:644–7.

    Article  Google Scholar 

  60. Hypothermia after Cardiac Arrest Study G. Mild therapeutic hypothermia to improve the neurologic outcome after cardiac arrest. N Engl J Med. 2002;346:549–56.

    Article  Google Scholar 

  61. Bastiaenen R, Hedley PL, Christiansen M, Behr ER. Therapeutic hypothermia and ventricular fibrillation storm in early repolarization syndrome. Heart Rhythm Off J Heart Rhythm Soc. 2010;7:832–4.

    Article  Google Scholar 

  62. Richter S, Ehrlich JR, Fassbender S, Fichtlscherer S. Malignant osborn waves during therapeutic hypothermia. Europace Eur Pacing Arrhythmias Card Electrophysiol J Work Groups Card Pacing, Arrhythmias Card Cell Electrophysiol Eur Soc Cardiol. 2009;11:668–9.

    Article  Google Scholar 

  63. Federman NJ, Mechulan A, Klein GJ, Krahn AD. Ventricular fibrillation induced by spontaneous hypothermia in a patient with early repolarization syndrome. J Cardiovasc Electrophysiol. 2013;24:586–8.

    Article  PubMed  Google Scholar 

  64. Lassnig E, Maurer E, Nomeyer R, Eber B. Osborn waves and incessant ventricular fibrillation during therapeutic hypothermia. Resuscitation. 2010;81:500–1.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Gi-Byoung Nam MD, PhD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Nam, GB. (2016). Clinical Features of Electrical Storms Associated with J Wave Syndromes and Acquired Forms of J Wave Syndrome. In: Antzelevitch, C., Yan, GX. (eds) J Wave Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-31578-2_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-31578-2_14

  • 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