Benign early repolarization

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| caption = Classic and new definitions of early repolarization with end-QRS notching and end-QRS slurring

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| frequency = 1-13%{{cite journal | vauthors = Bourier F, Denis A, Cheniti G, Lam A, Vlachos K, Takigawa M, Kitamura T, Frontera A, Duchateau J, Pambrun T, Klotz N, Derval N, Sacher F, Jais P, Haissaguerre M, Hocini M | display-authors = 6 | title = Early Repolarization Syndrome: Diagnostic and Therapeutic Approach | journal = Frontiers in Cardiovascular Medicine | volume = 5 | pages = 169 | date = 2018-11-27 | pmid = 30542653 | pmc = 6278243 | doi = 10.3389/fcvm.2018.00169 | doi-access = free }}

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| caption2 = ECG of a benign early repolarization in a 15-year-old male

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Benign early repolarization (BER) or early repolarization is found on an electrocardiogram (ECG) in about 1% of those with chest pain. It is diagnosed based on an elevated J-point / ST elevation with an end-QRS notch or end-QRS slur and where the ST segment concave up. It is believed to be a normal variant.{{cite journal | vauthors = Brady WJ, Chan TC | title = Electrocardiographic manifestations: benign early repolarization | journal = The Journal of Emergency Medicine | volume = 17 | issue = 3 | pages = 473–478 | year = 1999 | pmid = 10338242 | doi = 10.1016/S0736-4679(99)00010-4 }}

Benign early repolarization that occurs as some patterns is associated with ventricular fibrillation. The association, revealed by research performed in the late 2000s, is very small.

Types

Benign early repolarization, very prevalent in younger people and healthy male athletes, can be divided into 3 subtypes:{{Cite web| vauthors = Buttner R, Cadogan M |date=2021-01-21|title='Benign' Early Repolarization Misnomer|url=https://litfl.com/le-syndrome-dhaissaguerre/|access-date=2021-12-25|website=Life in the Fast Lane • LITFL|language=en-US}}{{cite journal | vauthors = Antzelevitch C, Yan GX, Viskin S | title = Rationale for the use of the terms J-wave syndromes and early repolarization | journal = Journal of the American College of Cardiology | volume = 57 | issue = 15 | pages = 1587–1590 | date = April 2011 | pmid = 21474038 | pmc = 3073061 | doi = 10.1016/j.jacc.2010.11.038 }}

  • Type 1 – BER pattern seen in lateral precordial leads.
  • Type 2 – BER pattern seen in inferior or inferolateral leads.
  • Type 3 – BER pattern seen globally (inferior, lateral, right precordial leads).

Associations with serious conditions

Research in the late 2000s has linked this finding to ventricular fibrillation, particularly in those who have fainted or have a family history of sudden cardiac death.{{cite journal | vauthors = Stern S | title = Clinical aspects of the early repolarization syndrome: a 2011 update | journal = Annals of Noninvasive Electrocardiology | volume = 16 | issue = 2 | pages = 192–195 | date = April 2011 | pmid = 21496171 | pmc = 6932469 | doi = 10.1111/j.1542-474X.2011.00429.x }}{{cite journal | vauthors = Haïssaguerre M, Derval N, Sacher F, Jesel L, Deisenhofer I, de Roy L, Pasquié JL, Nogami A, Babuty D, Yli-Mayry S, De Chillou C, Scanu P, Mabo P, Matsuo S, Probst V, Le Scouarnec S, 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, Clémenty J | display-authors = 6 | title = Sudden cardiac arrest associated with early repolarization | journal = The New England Journal of Medicine | volume = 358 | issue = 19 | pages = 2016–2023 | date = May 2008 | pmid = 18463377 | doi = 10.1056/NEJMoa071968 | doi-access = free }}{{cite journal | vauthors = Nam GB, Kim YH, Antzelevitch C | title = Augmentation of J waves and electrical storms in patients with early repolarization | journal = The New England Journal of Medicine | volume = 358 | issue = 19 | pages = 2078–2079 | date = May 2008 | pmid = 18463391 | pmc = 2515862 | doi = 10.1056/NEJMc0708182 }} Although there is a significant relationship between ventricular fibrillation and some early repolarization's patterns, the overall lifetime occurrence of idiopathic ventricular fibrillation is exceptionally rare.{{cite journal | vauthors = Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, Blom N, Brugada J, Chiang CE, Huikuri H, Kannankeril P, Krahn A, Leenhardt A, Moss A, Schwartz PJ, Shimizu W, Tomaselli G, Tracy C, Ackerman M, Belhassen B, Estes NA, Fatkin D, Kalman J, Kaufman E, Kirchhof P, Schulze-Bahr E, Wolpert C, Vohra J, Refaat M, Etheridge SP, Campbell RM, Martin ET, Quek SC | display-authors = 6 | title = Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes | journal = Europace | volume = 15 | issue = 10 | pages = 1389–1406 | date = October 2013 | pmid = 23994779 | doi = 10.1093/europace/eut272 }} There has also been an association between early repolarization and short QT syndrome.{{cite journal | vauthors = Watanabe H, Makiyama T, Koyama T, Kannankeril PJ, Seto S, Okamura K, Oda H, Itoh H, Okada M, Tanabe N, Yagihara N, Kamakura S, Horie M, Aizawa Y, Shimizu W | display-authors = 6 | title = High prevalence of early repolarization in short QT syndrome | journal = Heart Rhythm | volume = 7 | issue = 5 | pages = 647–652 | date = May 2010 | pmid = 20206319 | doi = 10.1016/j.hrthm.2010.01.012 }}

= Risk factors =

  • Male gender{{cite journal | vauthors = Junttila MJ, Sager SJ, Tikkanen JT, Anttonen O, Huikuri HV, Myerburg RJ | title = Clinical significance of variants of J-points and J-waves: early repolarization patterns and risk | journal = European Heart Journal | volume = 33 | issue = 21 | pages = 2639–2643 | date = November 2012 | pmid = 22645193 | doi = 10.1093/eurheartj/ehs110 }}
  • J-point and horizontal or descending / downsloping ST segment (especially in inferior leads){{cite journal | vauthors = Mercer BN, Begg GA, Page SP, Bennett CP, Tayebjee MH, Mahida S | title = Early Repolarization Syndrome; Mechanistic Theories and Clinical Correlates | journal = Frontiers in Physiology | volume = 7 | pages = 266 | date = 2016-06-30 | pmid = 27445855 | pmc = 4927622 | doi = 10.3389/fphys.2016.00266 | doi-access = free }}{{Cite journal|last1=Sethi|first1=Kamal K.|last2=Sethi|first2=Kabir|last3=Chutani|first3=Surendra K.|date=2014|title=Early repolarisation and J wave syndromes|journal=Indian Heart Journal|volume=66|issue=4|pages=443–452|doi=10.1016/j.ihj.2014.06.002|issn=0019-4832|pmc=4150048|pmid=25173204}}
  • Elevation of ST segment by 2 mm
  • Elevation of a J-wave by 0.2 mV or more{{cite journal | vauthors = Sayedahmad Z, Darmoch F, Al-Khadra Y, Kabach A, Alraies MC | title = Does early repolarization on ECG increase the risk of cardiac death in healthy people? | journal = Cleveland Clinic Journal of Medicine | volume = 86 | issue = 3 | pages = 165–166 | date = March 2019 | pmid = 30849033 | doi = 10.3949/ccjm.86a.17032 | s2cid = 73467268 | doi-access = free }}
  • J-point distribution globally
  • QRS longer than 110 ms
  • Longer duration of J wave, more than 60 ms{{Cite web|title=Early Repolarization Syndrome|url=https://www.acc.org/latest-in-cardiology/articles/2016/05/16/14/51/http%3a%2f%2fwww.acc.org%2flatest-in-cardiology%2farticles%2f2016%2f05%2f16%2f14%2f51%2fearly-repolarization-syndrome|access-date=2021-12-25|website=American College of Cardiology}}

Electrocardiography

On an electrocardiogram (EKG or ECG), benign early repolarization may produce an elevation of the J-point and ST segment in 2 or more leads, similar to that observed in heart attacks (myocardial infarction). However, with benign early repolarization, the ST segment is usually concave up, rather than concave down (as with heart attacks), and there is a notable absence of reciprocal changes suggestive of ischemia on the EKG.

Causes

It is thought that the mechanism causing early repolarization is a more excitable ion channel system, which causes a faster myocardium contraction. Studies have shown that higher testosterone levels in males result in an increased outward potassium currents causing J-point elevation.{{Cite journal|last1=Bourier|first1=Felix|last2=Denis|first2=Arnaud|last3=Cheniti|first3=Ghassen|last4=Lam|first4=Anna|last5=Vlachos|first5=Konstantinos|last6=Takigawa|first6=Masateru|last7=Kitamura|first7=Takeshi|last8=Frontera|first8=Antonio|last9=Duchateau|first9=Josselin|last10=Pambrun|first10=Thomas|last11=Klotz|first11=Nicolas|date=2018|title=Early Repolarization Syndrome: Diagnostic and Therapeutic Approach|journal=Frontiers in Cardiovascular Medicine|volume=5|pages=169|doi=10.3389/fcvm.2018.00169|pmid=30542653|pmc=6278243|issn=2297-055X|doi-access=free}}

Epidemiology

Benign early repolarization occurs in about 1 to 13 percent of the general population with a significant increase in occurrence within athletes and adolescents.{{cite journal | vauthors = Bourier F, Denis A, Cheniti G, Lam A, Vlachos K, Takigawa M, Kitamura T, Frontera A, Duchateau J, Pambrun T, Klotz N, Derval N, Sacher F, Jais P, Haissaguerre M, Hocini M | display-authors = 6 | title = Early Repolarization Syndrome: Diagnostic and Therapeutic Approach | journal = Frontiers in Cardiovascular Medicine | volume = 5 | pages = 169 | date = 2018-11-27 | pmid = 30542653 | pmc = 6278243 | doi = 10.3389/fcvm.2018.00169 | doi-access = free }} In one study, an occurrence of early repolarization was observed in 31.6% of elite athletes while in another study occurrence was observed in 25.1% of athletes.{{cite journal | vauthors = Serra-Grima R, Doñate M, Álvarez-García J, Barradas-Pires A, Ferrero A, Carballeira L, Puig T, Rodríguez E, Cinca J | display-authors = 6 | title = Long-term follow-up of early repolarization pattern in elite athletes | journal = The American Journal of Medicine | volume = 128 | issue = 2 | pages = 192.e1–192.e9 | date = February 2015 | pmid = 24979742 | doi = 10.1016/j.amjmed.2014.06.017 | doi-access = free }}{{cite journal | vauthors = Noseworthy PA, Weiner R, Kim J, Keelara V, Wang F, Berkstresser B, Wood MJ, Wang TJ, Picard MH, Hutter AM, Newton-Cheh C, Baggish AL | display-authors = 6 | title = Early repolarization pattern in competitive athletes: clinical correlates and the effects of exercise training | journal = Circulation: Arrhythmia and Electrophysiology | volume = 4 | issue = 4 | pages = 432–440 | date = August 2011 | pmid = 21543642 | pmc = 3700366 | doi = 10.1161/CIRCEP.111.962852 }}

Being a male is strongly associated with early repolarization ECG pattern, and 70% of subjects with early repolarization are males. Prevalence of early repolarization declines in males from early adulthood until middle-age which could suggest a hormonal influence on its presence. Early repolarization patterns are more common in physically active younger individuals, athletes and Africans.{{cite journal | vauthors = Sethi KK, Sethi K, Chutani SK | title = Early repolarisation and J wave syndromes | journal = Indian Heart Journal | volume = 66 | issue = 4 | pages = 443–452 | date = 2014 | pmid = 25173204 | pmc = 4150048 | doi = 10.1016/j.ihj.2014.06.002 }}

Genetics

Genes associated with ER and ATP sensitive potassium current channel mutations are KCNJ8, ABCC9{{cite journal | vauthors = Haïssaguerre M, Chatel S, Sacher F, Weerasooriya R, Probst V, Loussouarn G, Horlitz M, Liersch R, Schulze-Bahr E, Wilde A, Kääb S, Koster J, Rudy Y, Le Marec H, Schott JJ | display-authors = 6 | title = Ventricular fibrillation with prominent early repolarization associated with a rare variant of KCNJ8/KATP channel | journal = Journal of Cardiovascular Electrophysiology | volume = 20 | issue = 1 | pages = 93–98 | date = January 2009 | pmid = 19120683 | doi = 10.1111/j.1540-8167.2008.01326.x | s2cid = 21002962 }}{{cite journal | vauthors = Medeiros-Domingo A, Tan BH, Crotti L, Tester DJ, Eckhardt L, Cuoretti A, Kroboth SL, Song C, Zhou Q, Kopp D, Schwartz PJ, Makielski JC, Ackerman MJ | display-authors = 6 | title = Gain-of-function mutation S422L in the KCNJ8-encoded cardiac K(ATP) channel Kir6.1 as a pathogenic substrate for J-wave syndromes | journal = Heart Rhythm | volume = 7 | issue = 10 | pages = 1466–1471 | date = October 2010 | pmid = 20558321 | pmc = 3049900 | doi = 10.1016/j.hrthm.2010.06.016 }} Others associated with transient outward potassium current - KCNE5, DPP10, L-type voltage gated calcium current - CACNA1C, CACNB2B, CACNA2D1, sodium current - SCN5A, SCN10A.

History

Early repolarization with ST segment elevation was first described in 1936 by R.A. Shipley and W.R. Hallaran in a study of 200 healthy 20–35 year old people.{{Cite journal| vauthors = Shipley RA, Hallaran W |date=1936-03-01|title=The four-lead electrocardiogram in two hundred normal men and women |journal=American Heart Journal |volume=11|issue=3|pages=325–345 |doi=10.1016/S0002-8703(36)90417-9 }}

References

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Further reading

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  • {{cite journal | vauthors = Macfarlane PW, Antzelevitch C, Haissaguerre M, Huikuri HV, Potse M, Rosso R, Sacher F, Tikkanen JT, Wellens H, Yan GX | display-authors = 6 | title = The Early Repolarization Pattern: A Consensus Paper | journal = Journal of the American College of Cardiology | volume = 66 | issue = 4 | pages = 470–477 | date = July 2015 | pmid = 26205599 | doi = 10.1016/j.jacc.2015.05.033 | s2cid = 205571235 | doi-access = free }}
  • {{cite journal | vauthors = Junttila MJ, Sager SJ, Tikkanen JT, Anttonen O, Huikuri HV, Myerburg RJ | title = Clinical significance of variants of J-points and J-waves: early repolarization patterns and risk | journal = European Heart Journal | volume = 33 | issue = 21 | pages = 2639–2643 | date = November 2012 | pmid = 22645193 | doi = 10.1093/eurheartj/ehs110 }}

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