86. Mizumaki K, Nishida K, Iwamoto J, et al. Vagal activity modulates spontaneous augmentation of J-wave elevation in patients with idiopathic ventricular fibrillation. Heart Rhythm 2012; 9:249. 87. Letsas KP, Sacher F, Probst V, et al. Prevalence of early repolarization pattern in inferolateral leads in patients with Brugada syndrome. Heart Rhythm 2008; 5:1685. 88. Sarkozy A, Chierchia GB, Paparella G, et al. Inferior and lateral electrocardiographic repolarization abnormalities in Brugada syndrome. Circ Arrhythm Electrophysiol 2009; 2:154. https://www.uptodate.com/contents/early-repolarization/print 24/37 7/6/23, 3:25 PM Early repolarization - UpToDate 89. 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; 115:442. 90. Hong K, Berruezo-Sanchez A, Poungvarin N, et al. 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Al-Khatib SM, Stevenson WG, Ackerman MJ, 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:e91. 96. Viskin S, Belhassen B. Idiopathic ventricular fibrillation. Am Heart J 1990; 120:661. 97. Wever EF, Hauer RN, Oomen A, et al. Unfavorable outcome in patients with primary electrical disease who survived an episode of ventricular fibrillation. Circulation 1993; 88:1021. 98. Marcus FI. Idiopathic ventricular fibrillation. J Cardiovasc Electrophysiol 1997; 8:1075. 99. Survivors of out-of-hospital cardiac arrest with apparently normal heart. Need for definition and standardized clinical evaluation. Consensus Statement of the Joint Steering Committees of the Unexplained Cardiac Arrest Registry of Europe and of the Idiopathic Ventricular Fibrillation Registry of the United States. Circulation 1997; 95:265. 100. Meissner MD, Lehmann MH, Steinman RT, et al. Ventricular fibrillation in patients without significant structural heart disease: a multicenter experience with implantable cardioverter- defibrillator therapy. J Am Coll Cardiol 1993; 21:1406. https://www.uptodate.com/contents/early-repolarization/print 25/37 7/6/23, 3:25 PM Early repolarization - UpToDate 101. Ha ssaguerre M, Sacher F, Nogami A, et al. Characteristics of recurrent ventricular fibrillation associated with inferolateral early repolarization role of drug therapy. J Am Coll Cardiol 2009; 53:612. 102. Perrin T, Guieu R, Koutbi L, et al. Theophylline as an adjunct to control malignant ventricular arrhythmia associated with early repolarization. Pacing Clin Electrophysiol 2018; 41:444. 103. 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Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 10: The Cardiac Channelopathies: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015; 66:2424. Topic 83199 Version 27.0 https://www.uptodate.com/contents/early-repolarization/print 26/37 7/6/23, 3:25 PM Early repolarization - UpToDate GRAPHICS Expert consensus recommendations regarding early repolarization from the HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes Expert consensus statement on diagnosis 1. ER syndrome is diagnosed in the presence of J-point elevation 1 mm in 2 contiguous inferior and/or lateral leads of a standard 12-lead ECG in a patient resuscitated from otherwise unexplained VF/polymorphic VT. 2. ER syndrome can be diagnosed in an SCD victim with a negative autopsy and medical chart review with a previous ECG demonstrating J-point elevation 1 mm in 2 contiguous inferior and/or lateral leads of a standard 12-lead ECG. 3. ER pattern can be diagnosed in the presence of J-point elevation 1 mm in 2 contiguous inferior and/or lateral leads of a standard 12-lead ECG. Expert consensus statement on therapeutic intervention CLASS I 1. ICD implantation is recommended in patients with a diagnosis of ER syndrome who have survived a cardiac arrest. CLASS IIa 2. Isoproterenol infusion can be useful in suppressing electrical storms in patients with a diagnosis of ER syndrome. 3. Quinidine in addition to an ICD can be useful for secondary prevention of VF in patients with a diagnosis of ER syndrome. CLASS IIb 4. ICD implantation may be considered in symptomatic family members of ER syndrome patients with a history of syncope in the presence of ST-segment elevation >1 mm in 2 inferior or lateral leads. 5. ICD implantation may be considered in asymptomatic individuals who demonstrate a high-risk ER ECG pattern (high J-wave amplitude, horizontal/descending ST segment) in the presence of a strong family history of juvenile unexplained sudden death with or without a pathogenic mutation. CLASS III 6. ICD implantation is not recommended in asymptomatic patients with an isolated ER ECG pattern. ECG: electrocardiogram; VF: ventricular fibrillation; VT: ventricular tachycardia; ER: early repolarization; SCD: sudden cardiac death; ICD: implantable cardioverter defibrillator. https://www.uptodate.com/contents/early-repolarization/print 27/37 7/6/23, 3:25 PM Early repolarization - UpToDate Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes. Heart Rhythm 2013; 10:1932. Table used with the permission of Elsevier Inc. All rights reserved. Graphic 93392 Version 2.0 https://www.uptodate.com/contents/early-repolarization/print 28/37 7/6/23, 3:25 PM Early repolarization - UpToDate Early repolarization end-QRS notch and slur terminology (A) Illustration of the amplitudes J onset (Jo), J peak (Jp), and J termination (Jt), as well as durations D and D , in relation to an end-QRS notch, as defined in the text. 1 2 (B) Illustration of Jp and Jt, as well as D , in relation to an end-QRS slur. 2 Reproduced from: Macfarlane PW, Antzelevitch C, Haissaguerre M, et al. The Early Repolarization Pattern: A Consensus Paper. J Am Coll Cardiol 2015; 66:470. Illustration used with the permission of Elsevier Inc. All rights reserved. Graphic 118056 Version 1.0 https://www.uptodate.com/contents/early-repolarization/print 29/37 7/6/23, 3:25 PM Early repolarization - UpToDate Early repolarization 12 lead ECG Early repolarization manifest as inferior J-point slurring and lateral J-point notching, each >1 mm in two contiguous leads. Graphic 83883 Version 2.0 https://www.uptodate.com/contents/early-repolarization/print 30/37 7/6/23, 3:25 PM Early repolarization - UpToDate J or Osborn wave ECG change with hypothermia, including presence of J wave. Reproduced with permission from: Hickey R, De Caen A. Warming Procedures. In: Textbook of Pediatric Emergency Procedures, 2nd ed, King C, Henretig FM (Eds), Lippincott Williams & Wilkins, Philadelphia 2008. Copyright 2008 Lippincott Williams & Wilkins. www.lww.com. Graphic 56369 Version 11.0 https://www.uptodate.com/contents/early-repolarization/print 31/37 7/6/23, 3:25 PM Early repolarization - UpToDate Early repolarization 12-lead electrocardiogram Marked inferior and lateral ER of 3 mm. ER: early repolarization. Graphic 83891 Version 3.0 https://www.uptodate.com/contents/early-repolarization/print 32/37 7/6/23, 3:25 PM Early repolarization - UpToDate Action potentials in early repolarization A disproportionately abbreviated epicardial action potential compared with the endocardial action potential causes J-point elevation. Graphic 83884 Version 1.0 https://www.uptodate.com/contents/early-repolarization/print 33/37 7/6/23, 3:25 PM Early repolarization - UpToDate Action potential currents Major cardiac ion currents and channels responsible for a ventricular action potential are shown with their common name, abbreviation, and the gene and protein for the alpha subunit that forms the pore or transporter. The diagram on the left shows the time course of amplitude of each current during the action potential, but does not accurately reflect amplitudes relative to each of the other currents. This summary represents a ventricular myocyte, and lists only the major ion channels. The currents and their molecular nature vary within regions of the ventricles, and in atria, and other specialized cells such as nodal and Purkinje. Ion channels exist as part of multi-molecular complexes including beta subunits and other associated regulatory proteins which are also not shown. Courtesy of Jonathan C Makielski, MD, FACC. Graphic 70771 Version 4.0 https://www.uptodate.com/contents/early-repolarization/print 34/37 7/6/23, 3:25 PM Early repolarization - UpToDate Signal-averaged ECG in early repolarization Signal-averaged ECG in a patient with ER demonstrating normal parameters (filtered QRS duration 124 milliseconds, duration of high frequency low amplitude signal less than 40 microvolts of 37 milliseconds, and root mean square voltage in the terminal 40 milliseconds of 19 microvolts). Graphic 83890 Version 2.0 https://www.uptodate.com/contents/early-repolarization/print 35/37 7/6/23, 3:25 PM Early repolarization - UpToDate Approach for the acute management of ventricular arrhythmia An algorithmic approach for the acute management of ventricular arrhythmia associated with genetic disorders. VF: ventricular fibrillation; PMVT: polymorphic ventricular tachycardia, possible torsade de pointes; ERS: early repolarization syndrome. Refer to UpToDate text for details. Graphic 83895 Version 3.0 https://www.uptodate.com/contents/early-repolarization/print 36/37 7/6/23, 3:25 PM Early repolarization - UpToDate Contributor Disclosures Andrew Krahn, MD No relevant financial relationship(s) with ineligible companies to disclose. Manoj Obeyesekere, MBBS, MD No relevant financial relationship(s) with ineligible companies to disclose. Mark S Link, MD No relevant financial relationship(s) with ineligible companies to disclose. Nisha Parikh, MD, MPH No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. 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