J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge

Brugada, 1992, 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, 20, 1391, 10.1016/0735-1097(92)90253-J

Haissaguerre, 2008, Sudden cardiac arrest associated with early repolarization, N Engl J Med, 358, 2016, 10.1056/NEJMoa071968

Nam, 2008, Augmentation of J waves and electrical storms in patients with early repolarization, N Engl J Med, 358, 2078, 10.1056/NEJMc0708182

Rosso, 2008, J-point elevation in survivors of primary ventricular fibrillation and matched control subjects: incidence and clinical significance, J Am Coll Cardiol, 52, 1231, 10.1016/j.jacc.2008.07.010

Wilde, 2002, Proposed diagnostic criteria for the Brugada syndrome: consensus report, Circulation, 106, 2514, 10.1161/01.CIR.0000034169.45752.4A

Antzelevitch, 2005, Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association, Circulation, 111, 659, 10.1161/01.CIR.0000152479.54298.51

Priori, 2013, Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes, Heart Rhythm, 15, 1389

Priori, 2013, HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013, Heart Rhythm, 10, 1932, 10.1016/j.hrthm.2013.05.014

Priori, 2015, Eur Heart J, 36, 2757, 10.1093/eurheartj/ehv316

Clements, 1972, Diagnostic value of ECG abnormalities observed in subjects accidentally exposed to cold, Am J Cardiol, 29, 729, 10.1016/0002-9149(72)90178-6

Thompson, 1977, Evolutionary changes in the electrocardiogram of severe progressive hypothermia, J Electrocardiol, 10, 67, 10.1016/S0022-0736(77)80034-4

Eagle, 1994, Images in clinical medicine. Osborn waves of hypothermia, N Engl J Med, 10, 680, 10.1056/NEJM199403103301005

Kraus, 1920, Ueber die wirkung des kalziums auf den kreislauf 1, Dtsch Med Wochenschr, 46, 201, 10.1055/s-0029-1192473

Sridharan, 1984, Electrocardiographic J wave of hypercalcemia, Am J Cardiol, 54, 672, 10.1016/0002-9149(84)90273-X

Antzelevitch, 2010, J wave syndromes, Heart Rhythm, 7, 549, 10.1016/j.hrthm.2009.12.006

Wasserburger, 1961, The normal RS-T segment elevation variant, Am J Cardiol, 8, 184, 10.1016/0002-9149(61)90204-1

Mehta, 1995, Early repolarization on scalar electrocardiogram, Am J Med Sci, 309, 305, 10.1097/00000441-199506000-00001

Gussak, 2000, Early repolarization syndrome: clinical characteristics and possible cellular and ionic mechanisms, J Electrocardiol, 33, 299, 10.1054/jelc.2000.18106

Yan, 1999, Cellular basis for the Brugada syndrome and other mechanisms of arrhythmogenesis associated with ST segment elevation, Circulation, 100, 1660, 10.1161/01.CIR.100.15.1660

Shu, 2005, ST-segment elevation in the early repolarization syndrome, idiopathic ventricular fibrillation, and the Brugada syndrome: cellular and clinical linkage, J Electrocardiol, 38, 26, 10.1016/j.jelectrocard.2005.06.006

Antzelevitch, 2013, J wave syndromes: molecular and cellular mechanisms, J Electrocardiol, 46, 510, 10.1016/j.jelectrocard.2013.08.006

Mahida, 2015, History and clinical significance of early repolarization syndrome, Heart Rhythm, 12, 242, 10.1016/j.hrthm.2014.09.048

Wellens, 2014, Risk stratification for sudden cardiac death: current status and challenges for the futuredagger, Eur Heart J, 35, 1642, 10.1093/eurheartj/ehu176

Macfarlane, 2015, The early repolarization pattern: consensus paper, J Am Coll Cardiol, 66, 470, 10.1016/j.jacc.2015.05.033

Kanter, 2012, Brugada-like syndrome in infancy presenting with rapid ventricular tachycardia and intraventricular conduction delay, Circulation, 125, 14, 10.1161/CIRCULATIONAHA.111.054007

Antzelevitch, 2001, Molecular biology and cellular mechanisms of Brugada and long QT syndromes in infants and young children, J Electrocardiol, 34, 177, 10.1054/jelc.2001.28865

Wedekind, 2001, De novo mutation in the SCN5A gene associated with early onset of sudden infant death, Circulation, 104, 1158, 10.1161/hc3501.095361

Nagase, 2002, Epicardial electrogram of the right ventricular outflow tract in patients with the Brugada syndrome: using the epicardial lead, J Am Coll Cardiol, 39, 1992, 10.1016/S0735-1097(02)01888-0

Nakagawa, 2014, Left ventricular epicardial electrogram recordings in idiopathic ventricular fibrillation with inferior and lateral early repolarization, Heart Rhythm, 11, 314, 10.1016/j.hrthm.2013.10.057

Koncz, 2014, Mechanisms underlying the development of the electrocardiographic and arrhythmic manifestations of early repolarization syndrome, J Mol Cell Cardiol, 68C, 20, 10.1016/j.yjmcc.2013.12.012

Antzelevitch, 2006, Brugada syndrome, Pacing ClinElectrophysiol, 29, 1130, 10.1111/j.1540-8159.2006.00507.x

Coronel, 2005, Right ventricular fibrosis and conduction delay in a patient with clinical signs of Brugada syndrome: a combined electrophysiological, genetic, histopathologic, and computational study, Circulation, 112, 2769, 10.1161/CIRCULATIONAHA.105.532614

Bastiaenen, 2010, Therapeutic hypothermia and ventricular fibrillation storm in early repolarization syndrome, Heart Rhythm, 7, 832, 10.1016/j.hrthm.2010.02.037

Federman, 2013, Ventricular fibrillation induced by spontaneous hypothermia in a patient with early repolarization syndrome, J Cardiovasc Electrophysiol, 24, 586, 10.1111/jce.12030

Sakabe, 2003, Proportion and prognosis of healthy people with coved or saddle-back type ST segment elevation in the right precordial leads during 10 years follow-up, EurHeart J, 24, 1488, 10.1016/S0195-668X(03)00323-3

Tsuji, 2008, Prognosis of subjects with Brugada-type electrocardiogram in a population of middle-aged Japanese diagnosed during a health examination, Am J Cardiol, 102, 584, 10.1016/j.amjcard.2008.04.066

Gervacio-Domingo, 2008, The Brugada type 1 electrocardiographic pattern is common among Filipinos, J Clin Epidemiol, 61, 1067, 10.1016/j.jclinepi.2007.11.009

Ito, 2006, The prevalence and prognosis of a Brugada-type electrocardiogram in a population of middle-aged Japanese-American men with follow-up of three decades, Am J Med Sci, 331, 4, 10.1097/00000441-200601000-00008

Letsas, 2010, Tpeak-Tend interval and Tpeak-Tend/QT ratio as markers of ventricular tachycardia inducibility in subjects with Brugada ECG phenotype, Europace, 12, 271, 10.1093/europace/eup357

Gallagher, 2008, Prevalence and significance of Brugada-type ECG in 12,012 apparently healthy European subjects, Int J Cardiol, 130, 44, 10.1016/j.ijcard.2007.07.159

Pecini, 2010, The prevalence and relevance of the Brugada-type electrocardiogram in the Danish general population: data from the Copenhagen City Heart Study, Europace, 12, 982, 10.1093/europace/euq077

Patel, 2009, Prevalence of a Brugada pattern electrocardiogram in an urban population in the United States, Pacing Clin Electrophysiol, 32, 704, 10.1111/j.1540-8159.2009.02354.x

Lee, 2005, The incidence and prognosis of Brugada electrocardiographic pattern in the Manitoba Follow-Up Study, Can J Cardiol, 21, 1286

Tikkanen, 2009, Long-term outcome associated with early repolarization on electrocardiography, NEnglJ Med, 361, 2529, 10.1056/NEJMoa0907589

Sinner, 2010, 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, 7, e1000314, 10.1371/journal.pmed.1000314

Haruta, 2011, Incidence and prognostic value of early repolarization pattern in the 12–lead electrocardiogram, Circulation, 123, 2931, 10.1161/CIRCULATIONAHA.110.006460

Hayashi, 2015, The spectrum of epidemiology underlying sudden cardiac death, Circ Res, 116, 1887, 10.1161/CIRCRESAHA.116.304521

Brosnan, 2015, Early repolarization patterns associated with increased arrhythmic risk are common in young non-Caucasian Australian males and not influenced by athletic status, Heart Rhythm, 12, 1576, 10.1016/j.hrthm.2015.03.060

Priori, 2012, Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry, Journal of the American College of Cardiology, 59, 37, 10.1016/j.jacc.2011.08.064

Sroubek, 2016, Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled Analysis, Circulation, 10.1161/CIRCULATIONAHA.115.017885

Sangwatanaroj, 2001, Right ventricular electrocardiographic leads for detection of Brugada syndrome in sudden unexplained death syndrome survivors and their relatives, ClinCardiol, 24, 776

Miyamoto, 2007, Diagnostic and prognostic value of a type 1 Brugada electrocardiogram at higher (third or second) V1 to V2 recording in men with Brugada syndrome, Am J Cardiol, 99, 53, 10.1016/j.amjcard.2006.07.062

Nagase, 2010, Electroanatomical correlation of repolarization abnormalities in Brugada syndrome: detection of type 1 electrocardiogram in the right ventricular outflow tract, J Am Coll Cardiol, 56, 2143, 10.1016/j.jacc.2010.06.050

Veltmann, 2012, Insights into the location of type I ECG in patients with Brugada syndrome: correlation of ECG and cardiovascular magnetic resonance imaging, Heart Rhythm, 9, 414, 10.1016/j.hrthm.2011.10.032

Richter, 2010, Number of electrocardiogram leads displaying the diagnostic coved-type pattern in Brugada syndrome: a diagnostic consensus criterion to be revised, EurHeart J, 31, 1357, 10.1093/eurheartj/ehq049

Veerakul, 2012, Brugada syndrome: two decades of progress, Circ J, 76, 2713, 10.1253/circj.CJ-12-1352

Brugada, 2013, Brugada syndrome 1992–2012: 20 years of scientific excitement, and more, Eur Heart J, 34, 3610, 10.1093/eurheartj/eht113

Huikuri, 2015, Clinical aspects of inherited J-wave syndromes, Trends Cardiovasc Med, 25, 24, 10.1016/j.tcm.2014.08.008

Antzelevitch, 2015, J-wave syndromes: Brugada and early repolarization syndromes, Heart Rhythm, 12, 1852, 10.1016/j.hrthm.2015.04.014

Olde Nordkamp, 2015, Syncope in Brugada syndrome: prevalence, clinical significance, and clues from history taking to distinguish arrhythmic from nonarrhythmic causes, Heart Rhythm, 12, 367, 10.1016/j.hrthm.2014.10.014

Ikeda, 2006, The full stomach test as a novel diagnostic technique for identifying patients at risk for Brugada Syndrome, J Cardiovasc Electrophysiol, 17, 602, 10.1111/j.1540-8167.2006.00424.x

Shimeno, 2009, Usefulness of multichannel Holter ECG recording in the third intercostal space for detecting type 1 Brugada ECG: comparison with repeated 12–lead ECGs, J Cardiovasc Electrophysiol, 20, 1026, 10.1111/j.1540-8167.2009.01490.x

Viskin, 2015, Everybody has Brugada syndrome until proven otherwise?, Heart Rhythm, 12, 1595, 10.1016/j.hrthm.2015.04.017

Conte, 2014, Follow-up from childhood to adulthood of individuals with family history of Brugada syndrome and normal electrocardiograms, JAMA, 312, 2039, 10.1001/jama.2014.13752

Conte, 2014, Drug-induced Brugada syndrome in children: clinical features, device-based management and long-term follow-up, J Am Coll Cardiol, 63, 2272, 10.1016/j.jacc.2014.02.574

Gandjbakhch, 2014, Malignant response to ajmaline challenge in SCN5A mutation carriers: experience from a large familial study, Int J Cardiol, 172, 256, 10.1016/j.ijcard.2013.12.269

Kligfield, 2007, Journal of the American College of Cardiology, 49, 1109, 10.1016/j.jacc.2007.01.024

Papavassiliu, 2004, Magnetic resonance imaging findings in patients with Brugada syndrome, J Cardiovasc Electrophysiol, 15, 1133, 10.1046/j.1540-8167.2004.03681.x

Papavassiliu, 2010, Spontaneous type 1 electrocardiographic pattern is associated with cardiovascular magnetic resonance imaging changes in Brugada syndrome, Heart Rhythm, 7, 1790, 10.1016/j.hrthm.2010.09.004

Takagi, 2003, Abnormal response to sodium channel blockers in patients with Brugada syndrome: augmented localised wall motion abnormalities in the right ventricular outflow tract region detected by electron beam computed tomography, Heart, 89, 169, 10.1136/heart.89.2.169

Catalano, 2009, Magnetic resonance investigations in Brugada syndrome reveal unexpectedly high rate of structural abnormalities, EurHeart J, 30, 2241, 10.1093/eurheartj/ehp252

van Hoorn, 2012, SCN5A mutations in Brugada syndrome are associated with increased cardiac dimensions and reduced contractility, PLoS One, 7, e42037, 10.1371/journal.pone.0042037

Antzelevitch C, Brugada P, Brugada J, Brugada R, Nademanee K, Towbin JA. Clinical approaches to tachyarrhythmias. The Brugada syndrome. Vol 10. Armonk, NY: Futura Publishing Company, Inc. 1999

Antzelevitch, 2002, Brugada syndrome: historical perspectives and observations, EurHeart J, 23, 676, 10.1053/euhj.2001.3145

Slezak, 2009, Hibernating myocardium: pathophysiology, diagnosis, and treatment, Can J Physiol Pharmacol, 87, 252, 10.1139/Y09-011

Nademanee, 2015, Fibrosis, Connexin-43, and Conduction Abnormalities in the Brugada Syndrome, J Am Coll Cardiol, 66, 1976, 10.1016/j.jacc.2015.08.862

Corrado, 2006, Arrhythmogenic right ventricular cardiomyopathy/dysplasia: clinical impact of molecular genetic studies, Circulation, 113, 1634, 10.1161/CIRCULATIONAHA.105.616490

Corrado, 2011, Molecular biology and clinical management of arrhythmogenic right ventricular cardiomyopathy/dysplasia, Heart, 97, 530, 10.1136/hrt.2010.193276

Thiene, 1988, Right ventricular cardiomyopathy and sudden death in young people, The New England journal of medicine, 318, 129, 10.1056/NEJM198801213180301

Delmar, 2010, The cardiac desmosome and arrhythmogenic cardiomyopathies: from gene to disease, Circ Res, 107, 700, 10.1161/CIRCRESAHA.110.223412

Delmar, 2012, Cardiac connexins, mutations and arrhythmias, Curr OpinCardiol, 27, 236

Cerrone, 2012, Sodium current deficit and arrhythmogenesis in a murine model of plakophilin-2 haploinsufficiency, Cardiovasc Res, 95, 460, 10.1093/cvr/cvs218

Corrado, 2001, Right bundle branch block, right precordial ST-segment elevation, and sudden death in young people, Circulation, 103, 710, 10.1161/01.CIR.103.5.710

Cerrone, 2014, Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype, Circulation, 129, 1092, 10.1161/CIRCULATIONAHA.113.003077

Corrado, 2003, Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia, Circulation, 108, 3084, 10.1161/01.CIR.0000103130.33451.D2

Matsuo, 1999, The circadian pattern of the development of ventricular fibrillation in patients with Brugada syndrome, EurHeart J, 20, 465, 10.1053/euhj.1998.1332

Peters, 2004, Results of ajmaline testing in patients with arrhythmogenic right ventricular dysplasia-cardiomyopathy, Int J Cardiol, 95, 207, 10.1016/j.ijcard.2003.04.032

Peters, 2008, QRS fragmentation in standard ECG as a diagnostic marker of arrhythmogenic right ventricular dysplasia-cardiomyopathy, Heart Rhythm, 5, 1417, 10.1016/j.hrthm.2008.07.012

Postema PG, Wolpert C, Amin AS, Probst V, Borggrefe M, Roden DM, Priori SG, Tan HL, Hiraoka M, Brugada J, Wilde AA. Drugs and Brugada syndrome patients: review of the literature, recommendations, and an up-to-date website. 〈www.brugadadrugs.org〉. Heart Rhythm 2009;6:1335-1341

Shimizu, 2005, Acquired forms of the Brugada syndrome, J Electrocardiol, 38, 22, 10.1016/j.jelectrocard.2005.06.005

Brugada, 2000, Arrhythmia induction by antiarrhythmic drugs, Pacing Clin Electrophysiol, 23, 291, 10.1111/j.1540-8159.2000.tb06751.x

Brugada, 2000, Sodium channel blockers identify risk for sudden death in patients with ST-segment elevation and right bundle branch block but structurally normal hearts, Circulation, 101, 510, 10.1161/01.CIR.101.5.510

Miyazaki, 1996, Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome, J Am Coll Cardiol, 27, 1061, 10.1016/0735-1097(95)00613-3

Babaliaros, 2002, Tricyclic antidepressants and the Brugada syndrome: an example of Brugada waves appearing after the administration of desipramine, Clin Cardiol, 25, 395, 10.1002/clc.4950250809

Goldgran-Toledano, 2002, Overdose of cyclic antidepressants and the Brugada syndrome, N Engl J Med, 346, 1591, 10.1056/NEJM200205163462020

Tada, 2001, Brugada syndrome mimicked by tricyclic antidepressant overdose, J Cardiovasc Electrophysiol, 12, 10.1046/j.1540-8167.2001.00275.x

Ortega-Carnicer, 2001, Aborted sudden death, transient Brugada pattern, and wide QRS dysrrhythmias after massive cocaine ingestion, J Electrocardiol, 34, 345, 10.1054/jelc.2001.26318

Nogami, 2003, Enhancement of J-ST-segment elevation by the glucose and insulin test in Brugada syndrome, Pacing Clin Electrophysiol, 26, 332, 10.1046/j.1460-9592.2003.00044.x

Araki, 2003, Brugada syndrome with ventricular tachycardia and fibrillation related to hypokalemia, Circ J, 67, 93, 10.1253/circj.67.93

Pastor, 2001, Asymptomatic Brugada syndrome case unmasked during dimenhydrinate infusion, J Cardiovasc Electrophysiol, 12, 1192, 10.1046/j.1540-8167.2001.01192.x

Chiale, 2012, High-degree right bundle branch block obscuring the diagnosis of Brugada electrocardiographic pattern, Heart Rhythm, 9, 974, 10.1016/j.hrthm.2012.01.028

Baranchuk, 2012, Brugada phenocopy: new terminology and proposed classification, Ann Noninvasive Electrocardiol, 17, 299, 10.1111/j.1542-474X.2012.00525.x

Nam, 2012, Idiopathic ventricular fibrillation, early repolarization and other J wave-related ventricular fibrillation syndromes, Circ J, 76, 2723, 10.1253/circj.CJ-12-1306

Yan, 1996, Cellular basis for the electrocardiographic J wave, Circulation, 93, 372, 10.1161/01.CIR.93.2.372

McIntyre, 2012, Coexisting early repolarization pattern and Brugada syndrome: recognition of potentially overlapping entities, J Electrocardiol, 45, 195, 10.1016/j.jelectrocard.2011.10.008

Benito, 2008, Gender differences in clinical manifestations of Brugada syndrome, J Am Coll Cardiol, 52, 1567, 10.1016/j.jacc.2008.07.052

Kamakura, 2013, Significance of non-type 1 anterior early repolarization in patients with inferolateral early repolarization syndrome, J Am Coll Cardiol, 62, 1610, 10.1016/j.jacc.2013.05.081

Kawata, 2012, Effect of sodium-channel blockade on early repolarization in inferior/lateral leads in patients with idiopathic ventricular fibrillation and Brugada syndrome, Heart Rhythm, 9, 77, 10.1016/j.hrthm.2011.08.017

Matsumoto, 2003, Fundamental aspects of hypogonadism in the aging male, Rev Urol, 5, S3

Kalla, 2000, 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, 11, 95, 10.1111/j.1540-8167.2000.tb00743.x

Aizawa, 2012, 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, 59, 1948, 10.1016/j.jacc.2012.02.028

Nademanee, 1997, Sudden unexplained death syndrome in southeast Asia, Am J Cardiol, 79, 10, 10.1016/S0002-9149(97)00113-6

Watanabe, 2011, Electrocardiographic characteristics and SCN5A mutations in idiopathic ventricular fibrillation associated with early repolarization, Circ Arrhythm Electrophysiol, 4, 874, 10.1161/CIRCEP.111.963983

Antzelevitch, 2007, 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, 115, 442, 10.1161/CIRCULATIONAHA.106.668392

Shimizu, 2001, Catecholamines in children with congenital long QT syndrome and Brugada syndrome, J Electrocardiol, 34, 173, 10.1054/jelc.2001.28864

Suzuki, 2000, Infant case with a malignant form of Brugada syndrome, J Cardiovasc Electrophysiol, 11, 1277, 10.1046/j.1540-8167.2000.01277.x

Ohgo, 2007, Acute and chronic management in patients with Brugada syndrome associated with electrical storm of ventricular fibrillation, Heart Rhythm, 4, 695, 10.1016/j.hrthm.2007.02.014

Watanabe, 2006, Low-dose isoproterenol for repetitive ventricular arrhythmia in patients with Brugada syndrome, Eur Heart J, 27, 1579, 10.1093/eurheartj/ehl060

Hermida, 2004, Hydroquinidine therapy in Brugada syndrome, J Am Coll Cardiol, 43, 1853, 10.1016/j.jacc.2003.12.046

Belhassen, 2004, Efficacy of quinidine in high-risk patients with Brugada syndrome, Circulation, 110, 1731, 10.1161/01.CIR.0000143159.30585.90

Tsuchiya, 2002, Prevention of ventricular fibrillation by cilostazol, an oral phosphodiesterase inhibitor, in a patient with Brugada syndrome, J Cardiovasc Electrophysiol, 13, 698, 10.1046/j.1540-8167.2002.00698.x

Iguchi, 2013, Beneficial effects of cilostazol in a patient with recurrent ventricular fibrillation associated with early repolarization syndrome, Heart Rhythm, 10, 604, 10.1016/j.hrthm.2012.11.001

Agac, 2014, Conversion of Brugada type I to type III and successful control of recurrent ventricular arrhythmia with cilostazol, Arch Cardiovasc Dis, 107, 476, 10.1016/j.acvd.2012.06.008

Hasegawa, 2014, Long-term pharmacological therapy of Brugada syndrome: is J-wave attenuation a marker of drug efficacy?, Intern Med, 53, 1523, 10.2169/internalmedicine.53.1829

Shinohara, 2014, Combination therapy of cilostazol and bepridil suppresses recurrent ventricular fibrillation related to J-wave syndromes, Heart Rhythm, 11, 1441, 10.1016/j.hrthm.2014.05.001

Haissaguerre, 2009, Characteristics of recurrent ventricular fibrillation associated with inferolateral early repolarization role of drug therapy, J Am Coll Cardiol, 53, 612, 10.1016/j.jacc.2008.10.044

Junttila, 2014, Early repolarization as a predictor of arrhythmic and nonarrhythmic cardiac events in middle-aged subjects, Heart Rhythm, 11, 1701, 10.1016/j.hrthm.2014.05.024

Nam, 2010, Mode of onset of ventricular fibrillation in patients with early repolarization pattern vs. Brugada syndrome, Eur Heart J, 31, 330, 10.1093/eurheartj/ehp423

Nademanee, 2011, Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium, Circulation, 123, 1270, 10.1161/CIRCULATIONAHA.110.972612

Sacher, 2014, Insight into the mechanism of Brugada syndrome: epicardial substrate and modification during ajmaline testing, Heart Rhythm, 11, 732, 10.1016/j.hrthm.2013.05.023

Brugada, 2015, Brugada syndrome phenotype elimination by epicardial substrate ablation, Circ Arrhythm Electrophysiol, 8, 1373, 10.1161/CIRCEP.115.003220

Ghosh, 2010, Early repolarization associated with sudden death: insights from noninvasive electrocardiographic imaging, Heart Rhythm, 7, 534, 10.1016/j.hrthm.2009.12.005

Zhang, 2015, Cardiac electrophysiological substrate underlying the ECG phenotype and electrogram abnormalities in Brugada syndrome patients, Circulation, 131, 1950, 10.1161/CIRCULATIONAHA.114.013698

Kowalczyk, 2014, Giant J-wave and Brugada-like pattern in a patient with severe hypothermia, Acta Cardiol, 69, 66, 10.1080/AC.69.1.3011349

RuDusky, 2004, The electrocardiogram in hypothermia-the J wave and the Brugada syndrome, Am J Cardiol, 93, 671, 10.1016/j.amjcard.2003.10.065

Adler, 2013, Fever-induced Brugada pattern: how common is it and what does it mean?, Heart Rhythm, 10, 1375, 10.1016/j.hrthm.2013.07.030

Ansari, 2003, Profound hypothermia mimicking a Brugada type ECG, J Electrocardiol, 36, 257, 10.1016/S0022-0736(03)00043-8

Amin, 2008, Fever increases the risk for cardiac arrest in the Brugada syndrome, Ann Intern Med, 149, 216, 10.7326/0003-4819-149-3-200808050-00020

Rattanawong, 2016, Fever-induced Brugada syndrome is more common than previously suspected: a cross-sectional study from an endemic area, Ann Noninvasive Electrocardiol, 21, 136, 10.1111/anec.12288

Noda, 2003, Prominent J wave and ST segment elevation: serial electrocardiographic changes in accidental hypothermia, J Cardiovasc Electrophysiol, 14, 223, 10.1046/j.1540-8167.2003.02384.x

Tan, 2007, Lethal ECG changes hidden by therapeutic hypothermia, Lancet, 369, 78, 10.1016/S0140-6736(07)60034-8

Kurisu, 2008, Therapeutic hypothermia after out-of-hospital cardiac arrest due to Brugada syndrome, Resuscitation, 79, 332, 10.1016/j.resuscitation.2008.05.007

Patel, 2010, Early repolarization associated with ventricular arrhythmias in patients with chronic coronary artery disease, Circ Arrhythm Electrophysiol, 3, 489, 10.1161/CIRCEP.109.921130

Gurabi, 2014, Cellular mechanism underlying hypothermia-induced VT/VF in the setting of early repolarization and the protective effect of quinidine, cilostazol and milrinone, Circ Arrhythm Electrophysiol, 7, 134, 10.1161/CIRCEP.113.000919

Kawata, 2013, 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, 10, 1161, 10.1016/j.hrthm.2013.04.009

Antzelevitch, 2012, Genetic, molecular and cellular mechanisms underlying the J wave syndromes, Circ J, 76, 1054, 10.1253/circj.CJ-12-0284

Kapplinger, 2010, An international compendium of mutations in the SCN5A encoded cardiac sodium channel in patients referred for Brugada syndrome genetic testing, Heart Rhythm, 7, 33, 10.1016/j.hrthm.2009.09.069

Ackerman, 2011, HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA), Heart Rhythm, 8, 1308, 10.1016/j.hrthm.2011.05.020

Park, 2015, Genetically engineered SCN5A mutant pig hearts exhibit conduction defects and arrhythmias, J Clin Invest, 125, 403, 10.1172/JCI76919

Szel, 2014, Abnormal repolarization as the basis for late potentials and fractionated electrograms recorded from epicardium in experimental models of Brugada syndrome, J Am Coll Cardiol, 63, 2037, 10.1016/j.jacc.2014.01.067

Patocskai B, Szel T, Yoon N, Antzelevitch C. Cellular mechanisms underlying the fractionated and late potentials on epicardial electrograms and the ameliorative effect of epicardial radiofrequency ablation in an experimental model of Brugada syndrome. In: Program and Abstracts of the 24th Annual Upstate New York Cardiac Electrophysiology Society Meeting, November 3, 2014, Buffalo, NY, 2014Abstact 006.

Burashnikov, 2010, Mutations in the cardiac L-type calcium channel associated J wave sydnrome and sudden cardiac death, Heart Rhythm, 7, 1872, 10.1016/j.hrthm.2010.08.026

Cordeiro, 2009, Accelerated inactivation of the L-type calcium due to a mutation in CACNB2b due to a mutation in CACNB2b underlies Brugada syndrome, J Mol Cell Cardiol, 46, 695, 10.1016/j.yjmcc.2009.01.014

Antzelevitch, 2006, Loss-of-function mutations in the cardiac calcium channel underline a new clinical entity characterized by ST segment elevation, short QT intervals, and sudden cardiac death, Circ Res, 99, 1279

Gurnett, 1996, Dual function of the voltage-dependent Ca2+ channel alpha 2 delta subunit in current stimulation and subunit interaction, Neuron, 16, 431, 10.1016/S0896-6273(00)80061-6

Barajas-Martinez, 2012, Molecular genetic and functional association of Bugada and early repolarization syndromes with S422L missense mutation in KCNJ8, Heart Rhythm, 9, 548, 10.1016/j.hrthm.2011.10.035

Delaney, 2012, A KCNJ8 mutation associated with early repolarization and atrial fibrillation, Europace, 14, 1428, 10.1093/europace/eus150

Medeiros-Domingo, 2010, 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, 7, 1466, 10.1016/j.hrthm.2010.06.016

Hu, 2012, Novel mutations in the sodium channel 2 subunit gene (SCN2B) associated with Brugada syndrome and atrial fibrillation, Circulation, 126, A16521

Riuro, 2013, A missense mutation in the sodium channel β2 subunit reveals SCN2B as a new candidate gene for Brugada syndrome, Hum Mutat, 34, 961, 10.1002/humu.22328

Giudicessi, 2011, Transient outward current (Ito) gain-of-function mutations in the KCND3-encoded Kv4.3 potassium channel and Brugada syndrome, Heart Rhythm, 8, 1024, 10.1016/j.hrthm.2011.02.021

Delpón, 2008, Functional effects of KCNE3 mutation and its role in the development of Brugada syndrome, Circ Arrhythm Electrophysiol, 1, 209, 10.1161/CIRCEP.107.748103

Olesen, 2011, A novel nonsense variant in Nav1.5 cofactor MOG1 eliminates its sodium current increasing effect and may increase the risk of arrhythmias, Can J Cardiol, 27, 10.1016/j.cjca.2011.01.003

Kattygnarath, 2011, MOG1: a new susceptibility gene for Brugada syndrome, Circ Cardiovasc Genet, 4, 261, 10.1161/CIRCGENETICS.110.959130

Cerrone, 2013, Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype, Circulation, 129, 1092, 10.1161/CIRCULATIONAHA.113.003077

Hennessey, 2013, FGF12 is a candidate Brugada syndrome locus, Heart Rhythm, 10, 1886, 10.1016/j.hrthm.2013.09.064

Bezzina, 2013, Common variants at SCN5A-SCN10A and HEY2 are associated with Brugada syndrome, a rare disease with high risk of sudden cardiac death, Nat Genet, 45, 1044, 10.1038/ng.2712

Boukens, 2013, Reduced sodium channel function unmasks residual embryonic slow conduction in the adult right ventricular outflow tract, Circ Res, 113, 137, 10.1161/CIRCRESAHA.113.301565

Hartman, 2014, Myocardial deletion of transcription factor CHF1/Hey2 results in altered myocyte action potential and mild conduction system expansion but does not alter conduction system function or promote spontaneous arrhythmias, FASEB J, 28, 3007, 10.1096/fj.14-251728

Ishikawa, 2013, Novel SCN3B mutation associated with Brugada syndrome affects intracellular trafficking and function of Nav1.5, Circ J, 77, 959, 10.1253/circj.CJ-12-0995

Hu, 2009, A mutation in the beta 3 subunit of the cardiac sodium channel associated with Brugada ECG phenotype, Circ Cardiovasc Genet, 2, 270, 10.1161/CIRCGENETICS.108.829192

Watanabe, 2008, Sodium channel β1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans, J Clin Invest, 118, 2260

Valdivia, 2009, GPD1L links redox state to cardiac excitability by PKC-dependent phosphorylation of the sodium channel SCN5A, Am J Physiol Heart Circ Physiol, 297, H1446, 10.1152/ajpheart.00513.2009

Shy, 2013, Cardiac sodium channel NaV1.5 distribution in myocytes via interacting proteins: the multiple pool model, Biochim Biophys Acta, 1833, 886, 10.1016/j.bbamcr.2012.10.026

Weiss, 2002, Clinical and molecular heterogeneity in the Brugada syndrome: a novel gene locus on chromosome 3, Circulation, 105, 707, 10.1161/hc0602.103618

Hu, 2012, A novel rare variant in SCN1Bb linked to Brugada syndrome and SIDS by combined modulation of Na(v)1.5 and K(v)4.3 channel currents, Heart Rhythm, 9, 760, 10.1016/j.hrthm.2011.12.006

Hu, 2014, Mutations in SCN10A are responsible for a large fraction of cases of Brugada syndrome, J Am Coll Cardiol, 64, 66, 10.1016/j.jacc.2014.04.032

Behr, 2015, Role of common and rare variants in SCN10A: results from the Brugada syndrome QRS locus gene discovery collaborative study, Cardiovasc Res, 106, 520, 10.1093/cvr/cvv042

Antzelevitch, 2005, Cardiac repolarization. The long and short of it, Europace, 7, 3, 10.1016/j.eupc.2005.05.010

Liu, 2013, Molecular genetics and functional anomalies in a series of 248 Brugada cases with 11 mutations in the TRPM4 channel, PLoS One, 8, e54131, 10.1371/journal.pone.0054131

Perrin, 2014, 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, 7, 782, 10.1161/CIRCGENETICS.114.000623

Verkerk, 2005, Role of sequence variations in the human ether-a-go-go-related gene (HERG, KCNH2) in the Brugada syndrome 1, Cardiovasc Res, 68, 441, 10.1016/j.cardiores.2005.06.027

Wilders, 2010, Role of the R1135H KCNH2 mutation in Brugada syndrome, Int J Cardiol, 144, 149, 10.1016/j.ijcard.2008.12.177

Ohno, 2011, KCNE5 (KCNE1L) variants are novel modulators of Brugada syndrome and idiopathic ventricular fibrillation, Circ Arrhythm Electrophysiol, 4, 352, 10.1161/CIRCEP.110.959619

Boczek, 2014, Characterization of SEMA3A-encoded semaphorin as a naturally occurring Kv4.3 protein inhibitor and its contribution to Brugada syndrome, Circ Res, 115, 460, 10.1161/CIRCRESAHA.115.303657

Ueda, 2004, Functional characterization of a trafficking-defective HCN4 mutation, D553N, associated with cardiac arrhythmia, J Biol Chem, 279, 27194, 10.1074/jbc.M311953200

Noseworthy, 2011, The early repolarization pattern in the general population clinical correlates and heritability, J Am Coll Cardiol, 57, 2284, 10.1016/j.jacc.2011.04.003

Reinhard, 2011, Heritability of early repolarization: a population-based study, Circ Cardiovasc Genet, 4, 134, 10.1161/CIRCGENETICS.110.958298

Nunn, 2011, Prevalence of J-point elevation in sudden arrhythmic death syndrome families, J Am Coll Cardiol, 58, 286, 10.1016/j.jacc.2011.03.028

Haissaguerre, 2009, Ventricular fibrillation with prominent early repolarization associated with a rare variant of KCNJ8/KATP channel, J Cardiovasc Electrophysiol, 20, 93, 10.1111/j.1540-8167.2008.01326.x

Schwartz, 2013, Impact of genetics on the clinical management of channelopathies, J Am Coll Cardiol, 62, 169, 10.1016/j.jacc.2013.04.044

Raffan, 2011, Next generation sequencing: implications for clinical practice, Br Med Bull, 99, 53, 10.1093/bmb/ldr029

Refsgaard, 2012, High prevalence of genetic variants previously associated with LQT syndrome in new exome data, Eur J Hum Genet, 20, 905, 10.1038/ejhg.2012.23

Kapa, 2009, Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants, Circulation, 120, 1752, 10.1161/CIRCULATIONAHA.109.863076

Andreasen, 2013, Mutations in genes encoding cardiac ion channels previously associated with sudden infant death syndrome (SIDS) are present with high frequency in new exome data, Can J Cardiol, 29, 1104, 10.1016/j.cjca.2012.12.002

Kapplinger, 2010, An international compendium of mutations in the SCN5A-encoded cardiac sodium channel in patients referred for Brugada syndrome genetic testing, Heart Rhythm, 7, 33, 10.1016/j.hrthm.2009.09.069

Hu, 2014, ABCC9 is a novel Brugada and early repolarization syndrome susceptibility gene, Int J Cardiol, 171, 431, 10.1016/j.ijcard.2013.12.084

Alfares, 2015, Results of clinical genetic testing of 2,912 probands with hypertrophic cardiomyopathy: expanded panels offer limited additional sensitivity, Genet Med, 17, 8808, 10.1038/gim.2014.205

Le Scouarnec, 2015, Testing the burden of rare variation in arrhythmia-susceptibility genes provides new insights into molecular diagnosis for Brugada syndrome, Hum Mol Genet, 24, 2757, 10.1093/hmg/ddv036

Veeramah, 2014, The KCNJ8–S422L variant previously associated with J-wave syndromes is found at an increased frequency in Ashkenazi Jews, Eur J Hum Genet, 22, 94, 10.1038/ejhg.2013.78

Risgaard, 2013, High prevalence of genetic variants previously associated with Brugada syndrome in new exome data, Clin Genet, 84, 489, 10.1111/cge.12126

Campuzano, 2015, Determining the pathogenicity of genetic variants associated with cardiac channelopathies, Sci Rep, 5, 7953, 10.1038/srep07953

Crotti, 2012, Spectrum and prevalence of mutations Involving BrS1– through BrS12–susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing, J Am Coll Cardiol, 60, 1410, 10.1016/j.jacc.2012.04.037

Song, 2012, Cardiac sodium channel Nav1.5 mutations and cardiac arrhythmia, Pediatr Cardiol, 33, 943, 10.1007/s00246-012-0303-y

Ruklisa, 2015, Bayesian models for syndrome- and gene-specific probabilities of novel variant pathogenicity, Genome medicine, 7, 5, 10.1186/s13073-014-0120-4

Juang, 2014, Utilizing multiple in silico analyses to identify putative causal SCN5A variants in Brugada syndrome, Sci Rep, 4, 3850, 10.1038/srep03850

Walsh, 2014, Paralogue annotation identifies novel pathogenic variants in patients with Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia, J Med Genet, 51, 35, 10.1136/jmedgenet-2013-101917

Kapplinger, 2012, Enhanced classification of non-synonymous single nucleotide variants in the SCN5A-encoded Nav1.5 cardiac sodium channel, Heart Rhythm, 9, 1912, 10.1016/j.hrthm.2012.09.098

Kapplinger, 2015, Enhanced classification of Brugada syndrome-associated and long-QT syndrome-associated genetic variants in the SCN5A-encoded Nav1.5 cardiac sodium channel, Circ Cardiovasc Genet, 8, 582, 10.1161/CIRCGENETICS.114.000831

Probst, 2009, SCN5A mutations and the role of genetic background in the pathophysiology of Brugada syndrome, Circ Cardiovasc Genet, 2, 552, 10.1161/CIRCGENETICS.109.853374

Matsuo, 2003, Disappearance of the Brugada-type electrocardiogram after surgical castration: a role for testosterone and an explanation for the male preponderance?, Pacing Clin Electrophysiol, 26, 1151, 10.1046/j.1460-9592.2003.t01-1-00227.x

Antzelevitch, 2003, Androgens and male predominance of the Brugada syndrome phenotype, Pacing Clin Electrophysiol, 26, 1429, 10.1046/j.1460-9592.2003.t01-1-00206.x

Korte, 2015, Sudden cardiac arrest as a presentation of Brugada syndrome unmasked by thyroid storm, BMJ Case Rep, 2015

Wilde, 2010, The pathophysiological mechanism underlying Brugada syndrome: depolarization versus repolarization, J Mol Cell Cardiol, 49, 543, 10.1016/j.yjmcc.2010.07.012

Morita, 2009, Brugada syndrome: insights of ST elevation, arrhythmogenicity, and risk stratification from experimental observations, Heart Rhythm, 6, S34, 10.1016/j.hrthm.2009.07.018

Hoogendijk, 2010, The Brugada ECG pattern: a marker of channelopathy, structural heart disease, or neither? Toward a unifying mechanism of the Brugada syndrome, Circ Arrhythm Electrophysiol, 3, 283, 10.1161/CIRCEP.110.937029

Hoogendijk, 2010, Mechanism of right precordial ST-segment elevation in structural heart disease: excitation failure by current-to-load mismatch, Heart Rhythm, 7, 238, 10.1016/j.hrthm.2009.10.007

Patocskai, 2015, Expert opinion on orphan drugs, Expert Opin Orphan Drugs

Wilde, 2015, Bringing home the bacon? The next step in cardiac sodium channelopathies, J Clin Invest, 125, 99, 10.1172/JCI80014

Antzelevitch, 2002, Brugada syndrome: a decade of progress, Circ Res, 91, 1114, 10.1161/01.RES.0000046046.53721.90

Kurita, 2002, The electrophysiologic mechanism of ST-segment elevation in Brugada syndrome, J Am Coll Cardiol, 40, 330, 10.1016/S0735-1097(02)01964-2

Gurabi, 2014, Cellular mechanism underlying hypothermia-induced ventricular tachycardia/ventricular fibrillation in the setting of early repolarization and the protective effect of quinidine, cilostazol, and milrinone, Circ Arrhythm Electrophysiol, 7, 134, 10.1161/CIRCEP.113.000919

Huikuri, 2015, Separation of benign from ,alignant J waves, Heart Rhythm, 12, 384, 10.1016/j.hrthm.2014.11.019

Aizawa, 2015, Tachycardia-dependent augmentation of “notched J waves” in a general patient population without ventricular fibrillation or cardiac arrest: Not a repolarization but a depolarization abnormality?, Heart Rhythm, 12, 376, 10.1016/j.hrthm.2014.11.010

Badri, 2015, Cellular and ionic basis of J-wave syndromes, Trends Cardiovasc Med, 25, 12, 10.1016/j.tcm.2014.09.003

Morita, 2008, Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada syndrome, Circulation, 118, 1697, 10.1161/CIRCULATIONAHA.108.770917

Terho, 2014, Prevalence and prognostic significance of fragmented QRS complex in middle-aged subjects with and without clinical or electrocardiographic evidence of cardiac disease, Am J Cardiol, 114, 141, 10.1016/j.amjcard.2014.03.066

Osher, 1953, Electrocardiographic patern simulating acute myocardial injury, Am J Med Sci, 226, 541, 10.1097/00000441-195322650-00011

Viskin, 2014, Reply: is the J wave or the ST slope malignant…or neither?, J Am Coll Cardiol, 63, 1812, 10.1016/j.jacc.2013.10.072

Rosso, 2011, Risk of sudden death among young individuals with J waves and early repolarization: putting the evidence into perspective, Heart Rhythm, 8, 923, 10.1016/j.hrthm.2011.01.037

Aagaard, 2014, Prognostic value of automatically detected early repolarization, Am J Cardiol, 114, 1431, 10.1016/j.amjcard.2014.07.077

Uberoi, 2011, Early repolarization in an ambulatory clinical population, Circulation, 124, 2208, 10.1161/CIRCULATIONAHA.111.047191

Muramoto, 2014, Patterns and prognosis of all components of the J-wave pattern in multiethnic athletes and ambulatory patients, Am Heart J, 167, 259, 10.1016/j.ahj.2013.10.027

Perez, 2012, The prognostic value of early repolarization with ST-segment elevation in African Americans, Heart Rhythm, 9, 558, 10.1016/j.hrthm.2011.11.020

Klatsky, 2003, The early repolarization normal variant electrocardiogram: correlates and consequences, Am J Med, 115, 171, 10.1016/S0002-9343(03)00355-3

Rollin, 2012, Prevalence, prognosis, and identification of the malignant form of early repolarization pattern in a population-based study, Am J Cardiol, 110, 1302, 10.1016/j.amjcard.2012.06.033

Tikkanen, 2011, Early repolarization: electrocardiographic phenotypes associated with favorable long-term outcome, Circulation, 123, 2666, 10.1161/CIRCULATIONAHA.110.014068

Olson, 2011, Long-term prognosis associated with J-point elevation in a large middle-aged biracial cohort: the ARIC study, Eur Heart J, 32, 3098, 10.1093/eurheartj/ehr264

Rosso, 2012, J waves and early repolarization: do not confuse me with the facts!, Heart Rhythm, 9, 1603, 10.1016/j.hrthm.2012.07.019

Demidova, 2014, Transient and rapid QRS-widening associated with a J-wave pattern predicts impending ventricular fibrillation in experimental myocardial infarction, Heart Rhythm, 11, 1195, 10.1016/j.hrthm.2014.03.048

Shinde, 2007, Occurrence of “J waves” in 12–lead ECG as a marker of acute ischemia and their cellular basis, Pacing Clin Electrophysiol, 30, 817, 10.1111/j.1540-8159.2007.00760.x

Gourraud, 2013, Identification of large families in early repolarization syndrome, J Am Coll Cardiol, 61, 164, 10.1016/j.jacc.2012.09.040

Watanabe, 2010, High prevalence of early repolarization in short QT syndrome, Heart Rhythm, 7, 647, 10.1016/j.hrthm.2010.01.012

Letsas, 2012, Novel indexes of heterogeneity of ventricular repolarization in subjects with early repolarization pattern, Europace, 14, 877, 10.1093/europace/eur390

Rosso, 2012, Distinguishing “benign” from “malignant early repolarization”: The value of the ST-segment morphology, Heart Rhythm, 9, 225, 10.1016/j.hrthm.2011.09.012

Mahida, 2015, Role of electrophysiological studies in predicting risk of ventricular arrhythmia in early repolarization syndrome, J Am Coll Cardiol, 65, 151, 10.1016/j.jacc.2014.10.043

Probst, 2010, Long-term prognosis of patients diagnosed with Brugada syndrome: results from the FINGER Brugada Syndrome Registry, Circulation, 121, 635, 10.1161/CIRCULATIONAHA.109.887026

Kamakura, 2009, Long-term prognosis of probands with Brugada-pattern ST-elevation in leads V1–V3, Circ Arrhythm Electrophysiol, 2, 495, 10.1161/CIRCEP.108.816892

Conte, 2015, Implantable cardioverter-defibrillator therapy in Brugada syndrome: a 20-year single-center experience, J Am Coll Cardiol, 65, 879, 10.1016/j.jacc.2014.12.031

Sacher, 2013, Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study: part 2, Circulation, 128, 1739, 10.1161/CIRCULATIONAHA.113.001941

Priori, 2012, Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry, J Am Coll Cardiol, 59, 37, 10.1016/j.jacc.2011.08.064

Take, 2012, Identification of high-risk syncope related to ventricular fibrillation in patients with Brugada syndrome, Heart Rhythm, 9, 752, 10.1016/j.hrthm.2011.11.045

Conte, 2014, Clinical characteristics, management, and prognosis of elderly patients with Brugada syndrome, J Cardiovasc Electrophysiol, 25, 514, 10.1111/jce.12359

Priori, 2000, Brugada syndrome and sudden cardiac death in children, Lancet, 355, 808, 10.1016/S0140-6736(99)05277-0

Probst, 2007, Clinical aspects and prognosis of Brugada syndrome in children, Circulation, 115, 2042, 10.1161/CIRCULATIONAHA.106.664219

Meregalli, 2009, Type of SCN5A mutation determines clinical severity and degree of conduction slowing in loss-of-function sodium channelopathies, Heart Rhythm, 6, 341, 10.1016/j.hrthm.2008.11.009

Cordeiro, 2006, Compound heterozygous mutations P336L and I1660V in the human cardiac sodium channel associated with the Brugada syndrome, Circulation, 114, 2026, 10.1161/CIRCULATIONAHA.106.627489

Nunez, 2013, p.D1690N Nav1.5 rescues p.G1748D mutation gating defects in a compound heterozygous Brugada syndrome patient, Heart Rhythm, 10, 264, 10.1016/j.hrthm.2012.10.025

Poelzing, 2006, SCN5A polymorphism restores trafficking of a Brugada syndrome mutation on a separate gene, Circulation, 114, 368, 10.1161/CIRCULATIONAHA.105.601294

Viswanathan, 2003, A common SCN5A polymorphism modulates the biophysical effects of an SCN5A mutation, J Clin Invest, 111, 341, 10.1172/JCI200316879

Sommariva, 2013, Genetics can contribute to the prognosis of Brugada syndrome: a pilot model for risk stratification, EurJ Hum Genet, 21, 911, 10.1038/ejhg.2012.289

Delise, 2011, Risk stratification in individuals with the Brugada type 1 ECG pattern without previous cardiac arrest: usefulness of a combined clinical and electrophysiologic approach, Eur Heart J, 32, 169, 10.1093/eurheartj/ehq381

Yokokawa, 2010, Neurally mediated syncope as a cause of syncope in patients with Brugada electrocardiogram, J Cardiovasc Electrophysiol, 21, 186, 10.1111/j.1540-8167.2009.01599.x

Richter, 2009, Variability of the diagnostic ECG pattern in an ICD patient population with Brugada syndrome, J Cardiovasc Electrophysiol, 20, 69, 10.1111/j.1540-8167.2008.01282.x

Veltmann, 2006, A prospective study on spontaneous fluctuations between diagnostic and non-diagnostic ECGs in Brugada syndrome: implications for correct phenotyping and risk stratification, Eur Heart J, 27, 2544, 10.1093/eurheartj/ehl205

Rolf, 2003, The ajmaline challenge in Brugada syndrome: diagnostic impact, safety, and recommended protocol, Eur Heart J, 24, 1104, 10.1016/S0195-668X(03)00195-7

Veltmann, 2009, Response to intravenous ajmaline: a retrospective analysis of 677 ajmaline challenges, Europace, 11, 1345, 10.1093/europace/eup189

Okamura, 2015, Risk stratification in patients with Brugada syndrome without previous cardiac arrest: prognostic value of combined risk factors, Circ J, 79, 310, 10.1253/circj.CJ-14-1059

Govindan, 2010, Utility of high and standard right precordial leads during ajmaline testing for the diagnosis of Brugada syndrome, Heart, 96, 1904, 10.1136/hrt.2010.201244

Brugada, 2011, Electrophysiologic testing predicts events in Brugada syndrome patients, Heart Rhythm, 8, 1595, 10.1016/j.hrthm.2011.07.011

Wilde, 2011, EP testing does not predict cardiac events in Brugada syndrome, Heart Rhythm, 8, 1598, 10.1016/j.hrthm.2011.07.010

Viskin, 2007, Asymptomatic Brugada syndrome: a cardiac ticking time-bomb?, Europace, 9, 707, 10.1093/europace/eum174

Paul, 2007, Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data, Eur Heart J, 28, 2126, 10.1093/eurheartj/ehm116

Brugada, 2005, Patients with an asymptomatic Brugada electrocardiogram should undergo pharmacological and electrophysical testing, Circulation, 112, 279, 10.1161/CIRCULATIONAHA.104.485326

Sieira, 2015, Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience, Circ Arrhythm Electrophysiol, 8, 777, 10.1161/CIRCEP.114.002647

Makimoto, 2012, Clinical impact of the number of extrastimuli in programmed electrical stimulation in patients with Brugada type 1 electrocardiogram, Heart Rhythm, 9, 242, 10.1016/j.hrthm.2011.09.053

Tokioka, 2014, Electrocardiographic parameters and fatal Arrhythmic events in patients with Brugada syndrome: combination of depolarization and repolarization abnormalities, J Am Coll Cardiol, 63, 2131, 10.1016/j.jacc.2014.01.072

Take, 2012, Fragmented QRS: What is the meaning?, Indian Pacing Electrophysiol J, 12, 213, 10.1016/S0972-6292(16)30544-7

Kaneko, 2014, Electrical storm in patients with Brugada syndrome is associated with early repolarization, Circ Arrhythm Electrophysiol, 7, 1122, 10.1161/CIRCEP.114.001806

Takagi, 2013, Japan Idiopathic Ventricular Fibrillation Study I. The prognostic value of early repolarization (J wave) and ST-segment morphology after J wave in Brugada syndrome: multicenter study in Japan, Heart Rhythm, 10, 533, 10.1016/j.hrthm.2012.12.023

Rollin, 2013, Prevalence, characteristics, and prognosis role of type 1 ST elevation in the peripheral ECG leads in patients with Brugada syndrome, Heart Rhythm, 10, 1012, 10.1016/j.hrthm.2013.03.001

Marquez, 2005, Dynamic electrocardiographic changes after aborted sudden death in a patient with Brugada syndrome and rate-dependent right bundle branch block, J Electrocardiol, 38, 256, 10.1016/j.jelectrocard.2005.03.004

Kasanuki, 1997, Idiopathic ventricular fibrillation induced with vagal activity in patients without obvious heart disease, Circulation, 95, 2277, 10.1161/01.CIR.95.9.2277

Nademanee, 1997, Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men, Circulation, 96, 2595, 10.1161/01.CIR.96.8.2595

Ikeda, 2005, Noninvasive risk stratification of subjects with a Brugada-type electrocardiogram and no history of cardiac arrest, Ann Noninvasive Electrocardiol, 10, 396, 10.1111/j.1542-474X.2005.00055.x

Yoshioka, 2013, Identification of high-risk Brugada syndrome patients by combined analysis of late potential and T-wave amplitude variability on ambulatory electrocardiograms, Circ J, 77, 610, 10.1253/circj.CJ-12-0932

Tada, 2008, Clinical significance of macroscopic T-wave alternans after sodium channel blocker administration in patients with Brugada syndrome, J Cardiovasc Electrophysiol, 19, 56, 10.1111/j.1540-8167.2007.00967.x

Uchimura-Makita, 2014, Time-domain T-wave alternans is strongly associated with a history of ventricular fibrillation in patients with Brugada syndrome, J Cardiovasc Electrophysiol, 25, 1021, 10.1111/jce.12441

Junttila, 2008, Differences in 12–lead electrocardiogram between symptomatic and asymptomatic Brugada syndrome patients, J Cardiovasc Electrophysiol, 19, 380, 10.1111/j.1540-8167.2007.01050.x

Maury, 2013, Prevalence and prognostic role of various conduction disturbances in patients with the Brugada syndrome, Am J Cardiol, 11, 1384, 10.1016/j.amjcard.2013.06.033

Bigi, 2007, Clinical predictors of atrial fibrillation in Brugada syndrome, Europace, 9, 947, 10.1093/europace/eum110

Makimoto, 2010, Augmented ST-segment elevation during recovery from exercise predicts cardiac events in patients with Brugada syndrome, J Am Coll Cardiol, 56, 1576, 10.1016/j.jacc.2010.06.033

Castro Hevia, 2006, Tpeak-Tend and Tpeak-Tend dispersion as risk factors for ventricular tachycardia/ventricular fibrillation in patients with the Brugada syndrome, J Am Coll Cardiol, 47, 1828, 10.1016/j.jacc.2005.12.049

Karim Talib, 2012, Enhanced transmural dispersion of repolarization in patients with J wave syndromes, J Cardiovasc Electrophysiol, 23, 1109, 10.1111/j.1540-8167.2012.02363.x

Lambiase, 2010, Tpeak-Tend interval and Tpeak-Tend/QT ratio as markers of ventricular tachycardia inducibility in subjects with Brugada ECG phenotype, Europace, 12, 158, 10.1093/europace/eup424

Maury, 2015, Increased Tpeak-Tend interval is highly and independently related to arrhythmic events in Brugada syndrome, Heart Rhythm, 12, 2469, 10.1016/j.hrthm.2015.07.029

Sugao, 2005, Repolarization dynamics in patients with idiopathic ventricular fibrillation: pharmacological therapy with bepridil and disopyramide, J Cardiovasc Pharmacol, 45, 545, 10.1097/01.fjc.0000159660.16793.84

Aizawa, 2013, Efficacy and safety of bepridil for prevention of ICD shocks in patients with Brugada syndrome and idiopathic ventricular fibrillation, Int J Cardiol, 168, 5083, 10.1016/j.ijcard.2013.07.187

Calò, 2016, J Am Coll Cardiol, 67, 1427, 10.1016/j.jacc.2016.01.024

Masrur, 2015, Brugada syndrome, exercise, and exercise testing, Clin Cardiol, 38, 323, 10.1002/clc.22386

Amin, 2009, Exercise-induced ECG changes in Brugada syndrome, Circ Arrhythm Electrophysiol, 2, 531, 10.1161/CIRCEP.109.862441

Brugada, 2000, Pharmacological and device approach to therapy of inherited cardiac diseases associated with cardiac arrhythmias and sudden death, J Electrocardiol, 33, 41, 10.1054/jelc.2000.20322

Nademanee, 2003, Defibrillator Versus beta-Blockers for Unexplained Death in Thailand (DEBUT): a randomized clinical trial, Circulation, 107, 2221, 10.1161/01.CIR.0000066319.56234.C8

Sacher, 2013, Outcome after implantation of cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study—part 2, Circulation, 128, 1739, 10.1161/CIRCULATIONAHA.113.001941

Sacher, 2009, Remote implantable cardioverter defibrillator monitoring in a Brugada syndrome population, Europace, 11, 489, 10.1093/europace/eup034

De Maria, 2015, The entirely subcutaneous defibrillator (S-ICD): state of the art and selection of the ideal candidate, Curr Cardiol Rev, 11, 180, 10.2174/1573403X10666140827094126

Brugada, 1999, Use of the prophylactic implantable cardioverter defibrillator for patients with normal hearts, Am J Cardiol, 83, 98D, 10.1016/S0002-9149(98)01009-1

Takagi, 2007, Approach to the asymptomatic patients with Brugada syndrome, Indian Pacing Electrophysiol J, 7, 73

van Den Berg, 2001, Possible bradycardic mode of death and successful pacemaker treatment in a large family with features of long QT syndrome type 3 and Brugada syndrome, J Cardiovasc Electrophysiol, 12, 630, 10.1046/j.1540-8167.2001.00630.x

Bertomeu-Gonzalez, 2006, Syncopal monomorphic ventricular tachycardia with pleomorphism, sensitive to antitachycardia pacing in a patient with Brugada syndrome, Europace, 8, 1048, 10.1093/europace/eul117

Lee, 2000, Prevention of ventricular fibrillation by pacing in a man with Brugada syndrome, J Cardiovasc Electrophysiol, 11, 935, 10.1111/j.1540-8167.2000.tb00076.x

Cortez-Dias, 2014, Epicardial ablation for prevention of ventricular fibrillation in a patient with Brugada syndrome, Rev Port Cardiol, 33

Shah, 2011, Regional substrate ablation abolishes Brugada syndrome, J Cardiovasc Electrophysiol, 22, 1290, 10.1111/j.1540-8167.2011.02054.x

Brugada, 1998, Right bundle-branch block and ST-segment elevation in leads V1 through V3. A marker for sudden death in patients without demonstrable structural heart disease, Circulation, 97, 457, 10.1161/01.CIR.97.5.457

Chinushi, 1997, Discrepant drug action of disopyramide on ECG abnormalities and induction of ventricular arrhythmias in a patient with Brugada syndrome, J Electrocardiol, 30, 133, 10.1016/S0022-0736(97)80021-0

Minoura, 2012, Ionic and cellular mechanisms underlying the development of acquired Brugada syndrome in patients treated with antidepressants, J Cardiovasc Electrophysiol, 23, 423, 10.1111/j.1540-8167.2011.02196.x

Minoura, 2013, Effect of Wenxin Keli and quinidine to suppress arrhythmogenesis in an experimental model of Brugada syndrome, Heart Rhythm, 10, 1054, 10.1016/j.hrthm.2013.03.011

Johnson, 1960, Prevention of ventricular fibrillation during profound hypothermia by quinidine, Ann Surg, 151, 490, 10.1097/00000658-196004000-00007

Belhassen, 1987, Idiopathic ventricular fibrillation: inducibility and beneficial effects of class I antiarrhythmic agents, Circulation, 75, 809, 10.1161/01.CIR.75.4.809

Belhassen, 2002, The Brugada syndrome: is an implantable cardioverter defibrillator the only therapeutic option?, Pacing Clin Electrophysiol, 25, 1634, 10.1046/j.1460-9592.2002.01634.x

Alings, 2001, Quinidine induced electrocardiographic normalization in two patients with Brugada syndrome, Pacing Clin Electrophysiol, 24, 1420, 10.1046/j.1460-9592.2001.01420.x

Belhassen, 2004, Pharmacologic approach to therapy of Brugada syndrome: quinidine as an alternative to ICD therapy?, 202

Viskin, 2009, Empiric quinidine therapy for asymptomatic Brugada syndrome: time for a prospective registry, Heart Rhythm, 6, 401, 10.1016/j.hrthm.2008.11.030

Belhassen, 2009, Excellent long-term reproducibility of the electrophysiologic efficacy of quinidine in patients with idiopathic ventricular fibrillation and Brugada syndrome, Pacing Clin Electrophysiol, 32, 294, 10.1111/j.1540-8159.2008.02235.x

Marquez, 2013, Long-term efficacy of low doses of quinidine on malignant arrhythmias in Brugada syndrome with an implantable cardioverter-defibrillator: a case series and literature review, Heart Rhythm, 9, 1995, 10.1016/j.hrthm.2012.08.027

Pellegrino, 2013, Quinidine for the management of electrical storm in an old patient with Brugada syndrome and syncope, Acta Cardiol, 68, 201, 10.1080/AC.68.2.2967280

Probst, 2006, Progressive cardiac conduction defect is the prevailing phenotype in carriers of a Brugada syndrome SCN5A mutation, J Cardiovasc Electrophysiol, 17, 270, 10.1111/j.1540-8167.2006.00349.x

Schweizer, 2010, Successful acute and long-term management of electrical storm in Brugada syndrome using orciprenaline and quinine/quinidine, Clin Res Cardiol, 99, 467, 10.1007/s00392-010-0145-7

Marquez, 2005, Arrhythmic storm responsive to quinidine in a patient with Brugada syndrome and vasovagal syncope, Pacing Clin Electrophysiol, 28, 870, 10.1111/j.1540-8159.2005.00183.x

Viskin, 2007, Quinidine: a valuable medication joins the list of “endangered species”, Europace, 12, 1105, 10.1093/europace/eum181

Rosso, 2008, Outcome after implantation of cardioverter defribrillator in patients with Brugada syndrome: a multicenter Israeli study (ISRABRU), Isr Med Assoc J, 10, 435

Mok, 2004, Successful use of quinidine in treatment of electrical storm in Brugada syndrome, Pacing Clin Electrophysiol, 27, 821, 10.1111/j.1540-8159.2004.00537.x

Belhassen, 2015, Management of Brugada syndrome: a 33-year experience using electrophysiologically-guided therapy with Class 1A antiarrhythmic drugs, Circ Arrhythm Electrophysiol, 6, 1393, 10.1161/CIRCEP.115.003109

Bouzeman, 2014, Long-term follow-up of asymptomatic Brugada patients with inducible ventricular fibrillation under hydroquinidine, Europace, 16, 572, 10.1093/europace/eut279

Antzelevitch, 2001, The Brugada syndrome: ionic basis and arrhythmia mechanisms, J Cardiovasc Electrophysiol, 12, 268, 10.1046/j.1540-8167.2001.00268.x

Kyriazis, 2009, Electrical storm in Brugada syndrome successfully treated with orciprenaline: effect of low-dose quinidine on the electrocardiogram, Europace, 11, 665, 10.1093/europace/eup070

Shimizu, 2000, Body surface distribution and response to drugs of ST segment elevation in Brugada syndrome: clinical implication of eighty-seven-lead body surface potential mapping and its application to twelve-lead electrocardiograms, J Cardiovasc Electrophysiol, 11, 396, 10.1111/j.1540-8167.2000.tb00334.x

Tanaka, 2001, Successful prevention of recurrent ventricular fibrillation by intravenous isoproterenol in a patient with Brugada syndrome, Pacing Clin Electrophysiol, 24, 1293, 10.1046/j.1460-9592.2001.01293.x

Maury, 2004, Electrical storm in Brugada syndrome successfully treated using isoprenaline, Europace, 6, 130, 10.1016/j.eupc.2003.11.009

Maury, 2005, Electrical storms in Brugada syndrome: review of pharmacologic and ablative therapeutic options, Indian Pacing Electrophysiol J, 5, 25

Jongman, 2007, Electrical storms in Brugada syndrome successfully treated with isoproterenol infusion and quinidine orally, Neth Heart J, 15, 151, 10.1007/BF03085972

Sharif-Kazemi, 2011, Electrical storm in Brugada syndrome during pregnancy, Pacing Clin Electrophysiol, 34, e18, 10.1111/j.1540-8159.2010.02740.x

Furniss, 2012, Isoprenaline and quinidine to calm Brugada VF storm, BMJ Case Rep, 2013

Roten, 2012, Heterogeneous response of J wave syndromes to beta-adrenergic stimulation, Heart Rhythm, 9, 1970, 10.1016/j.hrthm.2012.08.003

Kanlop, 2011, Effects of cilostazol in the heart, J Cardiovasc Med (Hagerstown), 12, 88, 10.2459/JCM.0b013e3283439746

Bai, 2011, Cilostazol protects the heart against ischaemia reperfusion injury in a rabbit model of myocardial infarction: focus on adenosine, nitric oxide and mitochondrial ATP-sensitive potassium channels, Clin Exp Pharmacol Physiol, 38, 658, 10.1111/j.1440-1681.2011.05550.x

Abud, 2006, Failure of cilostazol in the prevention of ventricular fibrillation in a patient with Brugada syndrome, J Cardiovasc Electrophysiol, 17, 210, 10.1111/j.1540-8167.2005.00290.x

Szel, 2013, Cellular mechanisms underlying the effects of milrinone and cilostazol to supress arrhythmogenesis associated with Brugada syndrome, Heart Rhythm, 10, 1720, 10.1016/j.hrthm.2013.07.047

Murakami, 2010, Efficacy of low-dose bepridil for prevention of ventricular fibrillation in patients with Brugada syndrome with and without SCN5A mutation, J Cardiovasc Pharmacol, 56, 389, 10.1097/FJC.0b013e3181f03c2f

Kang, 2009, Bepridil up-regulates cardiac Na+ channels as a long-term effect by blunting proteasome signals through inhibition of calmodulin activity, Br J Pharmacol, 157, 404, 10.1111/j.1476-5381.2009.00174.x

Fish, 2006, Dimethyl lithospermate B, an extract of danshen, suppresses arrhythmogenesis associated with the Brugada syndrome, Circulation, 113, 1393, 10.1161/CIRCULATIONAHA.105.601690

Kanlop, 2010, Cilostazol attenuates ventricular arrhythmia induction and improves defibrillation efficacy in swine, Can J Physiol Pharmacol, 88, 422, 10.1139/Y09-127

Endoh, 1986, Effects of new inotropic agents on cyclic nucleotide metabolism and calcium transients in canine ventricular muscle, Circulation, 73, III117

Rapundalo, 1986, Myocardial actions of milrinone: characterization of its mechanism of action, Circulation, 73, III134

Atarashi, 1998, Chronotropic effects of cilostazol, a new antithrombotic agent, in patients with bradyarrhythmias, J Cardiovasc Pharmacol, 31, 534, 10.1097/00005344-199804000-00010

Matsui, 1999, Effects of pimobendan on the L-type Ca2+ current and developed tension in guinea-pig ventricular myocytes and papillary muscle: comparison with IBMX, milrinone, and cilostazol, Cardiovasc Drugs Ther, 13, 105, 10.1023/A:1007779908346

Aizawa, 2013, Electrical storm in idiopathic ventricular fibrillation is associated with early repolarization, J Am Coll Cardiol, 62, 1015, 10.1016/j.jacc.2013.05.030

Nakagawa, 2013, Left ventricular epicardial electrogram recordings in idiopathic ventricular fibrillation with inferior and lateral early repolarization, Heart Rhythm, 11, 314, 10.1016/j.hrthm.2013.10.057

Morita, 2007, Temperature modulation of ventricular arrhythmogenicity in a canine tissue model of Brugada syndrome, Heart Rhythm, 4, 188, 10.1016/j.hrthm.2006.10.015

Probst, 2010, Long-term prognosis of aatients diagnosed with Brugada syndrome: results from the FINGER Brugada Syndrome Registry, Circulation, 121, 635, 10.1161/CIRCULATIONAHA.109.887026

Takagi, 2013, The prognostic value of early repolarization (J wave) and ST-segment morphology after J wave in Brugada syndrome: multicenter study in Japan, Heart Rhythm, 10, 533, 10.1016/j.hrthm.2012.12.023

Brugada, 2003, Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome, Pacing Clin Electrophysiol, 26, 957

Huang, 2009, Role of signal-averaged electrocardiograms in arrhythmic risk stratification of patients with Brugada syndrome: a prospective study, Heart Rhythm, 6, 1156, 10.1016/j.hrthm.2009.05.007

Priori, 2002, Natural history of Brugada syndrome: insights for risk stratification and management, Circulation, 105, 1342, 10.1161/hc1102.105288

Brugada, 2003, Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest, Circulation, 108, 3092, 10.1161/01.CIR.0000104568.13957.4F

Cappato, 2010, J wave, QRS slurring and ST elevation in athletes with cardiac arrest in the absence of heart disease: marker of risk, or innocent bystander?, Circ Arrhythm Electrophysiol, 3, 305, 10.1161/CIRCEP.110.945824

Perez-Riera, 2012, “Benign” early repolarization versus malignant early abnormalities: clinical-electrocardiographic distinction and genetic basis, Cardiol J, 19, 337, 10.5603/CJ.2012.0063