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J wave syndrome: Benign or malignant?

Document Type : Review Article

Authors

1 Assistant Professor, Department of Cardiology, School of Medicine, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran

2 Associate Professor, Department of Cardiology, School of Medicine, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran

Abstract

J wave syndrome is an electrical disease of the heart due to pathologic early repolarization. It encompasses a clinical spectrum from aborted sudden cardiac death due to ventricular arrhythmia (VA) usually in young affected patients to self-terminating ventricular ectopies, and finally, asymptomatic relatives of probands detected during electrocardiography acquisition (early repolarization pattern). This syndrome consists of 2 phenotypes, early repolarization and Brugada syndrome. Herein, we first describe 2 patients with early repolarization and Brugada syndrome, then, discuss their definition, epidemiology, genetics, cellular mechanism, diagnosis, risk stratification, and finally, therapeutic challenges and options one by one in detail.

Keywords

References
  1. Antzelevitch C, Yan GX. J wave syndromes. Heart Rhythm 2010; 7(4): 549-58.
  2. Sethi KK, Sethi K, Chutani SK. Early repolarisation and J wave syndromes. Indian Heart J 2014; 66(4): 443-52.
  3. Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, et al. J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge. Heart Rhythm 2016; 13(10): e295-e324.
  4. Tomaszewski W. Electrocardiographic changes observed in a man frozen to death. Arch Mal Coeur 1938; 31: 525-8.
  5. Osborn JJ. Experimental hypothermia; respiratory and blood pH changes in relation to cardiac function. Am J Physiol 1953; 175(3): 389-98.
  6. Wasserburger RH, Alt WJ. The normal RS-T segment elevation variant. Am J Cardiol 1961; 8: 184-92.
  7. Macfarlane PW, Antzelevitch C, Haissaguerre M, Huikuri HV, Potse M, Rosso R, et al. The early repolarization pattern: A consensus paper. J Am Coll Cardiol 2015; 66(4): 470-7.
  8. Gussak I, Antzelevitch C. Early repolarization syndrome: Clinical characteristics and possible cellular and ionic mechanisms. J Electrocardiol 2000; 33(4): 299-309.
  9. Otto CM, Tauxe RV, Cobb LA, Greene HL, Gross BW, Werner JA, et al. Ventricular fibrillation causes sudden death in Southeast Asian immigrants. Ann Intern Med 1984; 101(1): 45-7.
  10. Brugada J, Brugada R, Brugada P. Pharmacological and device approach to therapy of inherited cardiac diseases associated with cardiac arrhythmias and sudden death. J Electrocardiol 2000; 33(Suppl): 41-7.
  11. Sethi KK, Sethi K, Chutani SK. J wave syndrome: Clinical diagnosis, risk stratification and treatment. J Atr Fibrillation 2014; 7(4): 1173.
  12. Mollazadeh R, Sehhati F, Eslami M, Nemati F, Monfarednasab M, Sefidbakht S. Characteristics of early repolarization pattern in the Iranian population. Iran Red Crescent Med J 2017; 19(3): e41081.
  13. Sinner MF, Reinhard W, Muller M, Beckmann BM, Martens E, Perz S, et al. Association of early repolarization pattern on ECG with risk of cardiac and all-cause mortality: A population-based prospective cohort study (MONICA/KORA). PLoS Med 2010; 7(7): e1000314.
  14. Mahida S, Derval N, Sacher F, Berte B, Yamashita S, Hooks DA, et al. History and clinical significance of early repolarization syndrome. Heart Rhythm 2015; 12(1): 242-9.
  15. Tikkanen JT, Anttonen O, Junttila MJ, Aro AL, Kerola T, Rissanen HA, et al. Long-term outcome associated with early repolarization on electrocardiography. N Engl J Med 2009; 361(26): 2529-37.
  16. Maury P, Rollin A. Prevalence of early repolarisation/J wave patterns in the normal population. J Electrocardiol 2013; 46(5): 411-6.
  17. Noseworthy PA, Tikkanen JT, Porthan K, Oikarinen L, Pietila A, Harald K, et al. The early repolarization pattern in the general population: Clinical correlates and heritability. J Am Coll Cardiol 2011; 57(22): 2284-9.
  18. Haissaguerre M, Nademanee K, Hocini M, Cheniti G, Duchateau J, Frontera A, et al. Depolarization versus repolarization abnormality underlying inferolateral J-wave syndromes: New concepts in sudden cardiac death with apparently normal hearts. Heart Rhythm 2019; 16(5): 781-90.
  19. Makarawate P, Chaosuwannakit N, Ruamcharoen Y, Panthongviriyakul A, Pongchaiyakul C, Tharaksa P, et al. Prevalence and associated factors of early repolarization pattern in healthy young northeastern Thai men: A correlation study with Brugada electrocardiography. J Arrhythm 2015; 31(4): 215-20.
  20. Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, 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.
  21. Gallagher MM, Forleo GB, Behr ER, Magliano G, De Luca L., Morgia V, et al. Prevalence and significance of Brugada-type ECG in 12,012 apparently healthy European subjects. Int J Cardiol 2008; 130(1): 44-8.
  22. Huikuri HV. Differences in characteristics between J-wave syndrome patients. Heart Rhythm 2017; 14(4): 562-3.
  23. Watanabe Y, Matsumoto A, Miki T, Seino S, Anzai N, Nakaya H. Electrophysiological analyses of transgenic mice overexpressing KCNJ8 with S422L mutation in cardiomyocytes. J Pharmacol Sci 2017; 135(1): 37-43.
  24. Medeiros-Domingo A, Tan BH, Crotti L, Tester DJ, Eckhardt L, Cuoretti A, et al. Gain-of-function mutation S422L in the KCNJ8-encoded cardiac K(ATP) channel Kir6.1 as a pathogenic substrate for J-wave syndromes. Heart Rhythm 2010; 7(10): 1466-71.
  25. Krogh Broendberg A., Pedersen LN, Nielsen JC, Jensen HK. Ankyrin-2 variants associated with idiopathic ventricular fibrillation storm in patients with intermittent early repolarization pattern. HeartRhythm Case Rep 2015; 1(5): 337-41.
  26. Antzelevitch C. J wave syndromes: Molecular and cellular mechanisms. J Electrocardiol 2013; 46(6): 510-8.
  27. Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, et al. 2017 AHA/ACC/HRS Guideline 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 on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol 2018; 72(14): e91-e220.
  28. Priori SG, Blomstrom-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, Camm J, et al. 2015 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.
  29. Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Europace 2013; 15(10): 1389-406.
  30. Koncz I, Gurabi Z, Patocskai B, Panama BK, Szel T, Hu D, et al. Mechanisms underlying the development of the electrocardiographic and arrhythmic manifestations of early repolarization syndrome. J Mol Cell Cardiol 2014; 68: 20-8.
  31. Chen M, Xu DZ, Wu AZ, Guo S, Wan J, Yin D, et al. Concomitant SK current activation and sodium current inhibition cause J wave syndrome. JCI Insight 2018; 3(22): e122329.
  32. Isbister JC, Krahn AD, Semsarian C, Sy RW. Brugada Syndrome: Clinical Care Amidst Pathophysiological Uncertainty. Heart Lung Circ 2020; 29(4): 538-46.
  33. Nakagawa E, Takagi M, Tatsumi H, Yoshiyama M. Successful radiofrequency catheter ablation for electrical storm of ventricular fibrillation in a patient with Brugada syndrome. Circ J 2008; 72(6): 1025-9.
  34. Yan GX, Antzelevitch C. Cellular basis for the Brugada syndrome and other mechanisms of arrhythmogenesis associated with ST-segment elevation. Circulation 1999; 100(15): 1660-6.
  35. Bourier F, Denis A, Cheniti G, Lam A, Vlachos K, Takigawa M, et al. Early repolarization syndrome: Diagnostic and therapeutic approach. Front Cardiovasc Med 2018; 5: 169.
  36. Kim SH, Nam GB, Yun SC, Choi HO, Choi KJ, Joung B, et al. Variants of Brugada Syndrome and Early Repolarization Syndrome: An Expanded Concept of J-Wave Syndrome. Pacing Clin Electrophysiol 2017; 40(2): 162-74.
  37. Kamakura T, Shinohara T, Yodogawa K, Murakoshi N, Morita H, Takahashi N, et al. Long-term prognosis of patients withJ-wave syndrome. Heart 2020; 106(4): 299-306.
  38. Yamaki M, Sato N, Imanishi R, Sakai H, Kawamura Y, Hasebe N. Low room temperature can trigger ventricular fibrillation in J wave syndromes. HeartRhythm Case Rep 2016; 2(4): 347-50.
  39. Rosso R, Kogan E, Belhassen B, Rozovski U, Scheinman MM, Zeltser D, et al. J-point elevation in survivors of primary ventricular fibrillation and matched control subjects: Incidence and clinical significance. J Am Coll Cardiol 2008; 52(15): 1231-8.
  40. Rosso R, Glikson E, Belhassen B, Katz A, Halkin A, Steinvil A, et al. Distinguishing "benign" from "malignant early repolarization": The value of the ST-segment morphology. Heart Rhythm 2012; 9(2): 225-9.
  41. Perrin MJ, Adler A, Green S, Al-Zoughool F, Doroshenko P, Orr N, et al. Evaluation of genes encoding for the transient outward current (Ito) identifies the KCND2 gene as a cause of J-wave syndrome associated with sudden cardiac death. Circ Cardiovasc Genet 2014; 7(6): 782-9.
  42. Di Stolfo G., Palumbo P, Castellana S, Mastroianno S, Biagini T, Palumbo O, et al. Sudden cardiac death in J wave syndrome with short QT associated to a novel mutation in Nav 1.8 coding gene SCN10A: First case report for a possible pharmacogenomic role. J Electrocardiol 2018; 51(5): 809-13.
  43. Priori SG, Gasparini M, Napolitano C, Della Bella P., Ottonelli AG, Sassone B, et al. Risk stratification in Brugada syndrome: Results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry. J Am Coll Cardiol 2012; 59(1): 37-45.
  44. Vohra J, Rajagopalan S. Update on the Diagnosis and Management of Brugada Syndrome. Heart Lung Circ 2015; 24(12): 1141-8.
  45. Letsas KP, Weber R, Astheimer K, Kalusche D, Arentz T. Tpeak-Tend interval and Tpeak-Tend/QT ratio as markers of ventricular tachycardia inducibility in subjects with Brugada ECG phenotype. Europace 2010; 12(2): 271-4.
  46. Sacher F, Probst V, Iesaka Y, Jacon P, Laborderie J, Mizon-Gerard F, et al. Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: A multicenter study. Circulation 2006; 114(22): 2317-24.
  47. Belhassen B, Viskin S, Antzelevitch C. The Brugada syndrome: Is an implantable cardioverter defibrillator the only therapeutic option? Pacing Clin Electrophysiol 2002; 25(11): 1634-40.
  48. Chinushi M, Aizawa Y, Ogawa Y, Shiba M, Takahashi K. Discrepant drug action of disopyramide on ECG abnormalities and induction of ventricular arrhythmias in a patient with Brugada syndrome. J Electrocardiol 1997; 30(2): 133-6.
  49. 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 2010; 99(7): 467-70.
  50. Shinohara T, Ebata Y, Ayabe R, Fukui A, Okada N, Yufu K, et al. Combination therapy of cilostazol and bepridil suppresses recurrent ventricular fibrillation related to J-wave syndromes. Heart Rhythm 2014; 11(8): 1441-5.
  51. Murakami M, Nakamura K, Kusano KF, Morita H, Nakagawa K, Tanaka M, et al. Efficacy of low-dose bepridil for prevention of ventricular fibrillation in patients with Brugada syndrome with and without SCN5A mutation. J Cardiovasc Pharmacol 2010; 56(4): 389-95.
  52. Szel T, Koncz I, Antzelevitch C. Cellular mechanisms underlying the effects of milrinone and cilostazol to suppress arrhythmogenesis associated with Brugada syndrome. Heart Rhythm 2013; 10(11): 1720-7.
  53. Bouzeman A, Traulle S, Messali A, Extramiana F, Denjoy I, Narayanan K, et al. Long-term follow-up of asymptomatic Brugada patients with inducible ventricular fibrillation under hydroquinidine. Europace 2014; 16(4): 572-7.
  54. Nademanee K, Veerakul G, Chandanamattha P, Chaothawee L, Ariyachaipanich A, Jirasirirojanakorn K, et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation 2011; 123(12): 1270-9.
ARYA Atherosclerosis Journal
Volume 17, Issue 1
January and February 2021
Pages 1-9
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