{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:27:47Z","timestamp":1790836067884,"version":"4.1.0"},"reference-count":114,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,1,4]],"date-time":"2023-01-04T00:00:00Z","timestamp":1672790400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cardiovasc. Med."],"abstract":"<jats:p>Inherited Arrhythmia Syndromes (IAS) including long QT and Brugada Syndrome, are characterized by life-threatening arrhythmias in the absence of apparent structural heart disease and are caused by pathogenic variants in genes encoding cardiac ion channels or associated proteins. Studies of large pedigrees of families affected by IAS have demonstrated incomplete penetrance and variable expressivity. Biological sex is one of several factors that have been recognized to modulate disease severity in IAS. There is a growing body of evidence linking sex hormones to the susceptibility to arrhythmias, yet, many sex-specific disease aspects remain underrecognized as female sex and women with IAS are underinvestigated and findings from male-predominant cohorts are often generalized to both sexes with minimal to no consideration of relevant sex-associated differences in prevalence, disease manifestations and outcome. In this review, we highlight current knowledge of sex-related biological differences in normal cardiac electrophysiology and sex-associated factors that influence IAS phenotypes.<\/jats:p>","DOI":"10.3389\/fcvm.2022.1010748","type":"journal-article","created":{"date-parts":[[2023,1,4]],"date-time":"2023-01-04T13:45:50Z","timestamp":1672839950000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":9,"title":["Sex-related differences in incidence, phenotype and risk of sudden cardiac death in inherited arrhythmia syndromes"],"prefix":"10.3389","volume":"9","author":[{"given":"Babken","family":"Asatryan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas S.","family":"Barth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,1,4]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"739","DOI":"10.1161\/CIRCULATIONAHA.120.048250","article-title":"Sex-related differences in cardiac channelopathies: implications for clinical practice","volume":"143","author":"Asatryan","year":"2021","journal-title":"Circulation."},{"key":"B2","doi-asserted-by":"publisher","first-page":"678","DOI":"10.1067\/mhj.2000.109918","article-title":"Sex differences on the electrocardiographic pattern of cardiac repolarization: possible role of testosterone","volume":"140","author":"Bidoggia","year":"2000","journal-title":"Am Heart J."},{"key":"B3","first-page":"690","article-title":"Sex differences in the evolution of the electrocardiographic QT interval with age","volume":"8","author":"Rautaharju","year":"1992","journal-title":"Can J Cardiol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"2107","DOI":"10.1016\/j.hrthm.2014.06.023","article-title":"How do sex hormones modify arrhythmogenesis in long QT syndrome? Sex hormone effects on arrhythmogenic substrate and triggered activity","volume":"11","author":"Odening","year":"2014","journal-title":"Heart Rhythm."},{"key":"B5","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1111\/j.1540-8159.2006.00407.x","article-title":"Influence of menstrual cycle on QT interval dynamics","volume":"29","author":"Nakagawa","year":"2006","journal-title":"Pacing Clin Electrophysiol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"1547","DOI":"10.1016\/S0735-1097(96)00351-8","article-title":"QT interval-heart rate relation during exercise in normal men and women: definition by linear regression analysis","volume":"28","author":"Kligfield","year":"1996","journal-title":"J Am Coll Cardiol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"868","DOI":"10.1161\/CIRCEP.111.962019","article-title":"Long-QT syndrome: from genetics to management","volume":"5","author":"Schwartz","year":"2012","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"418","DOI":"10.1161\/CIRCULATIONAHA.119.043132","article-title":"An International, multicentered, evidence-based reappraisal of genes reported to cause congenital Long QT Syndrome","volume":"141","author":"Adler","year":"2020","journal-title":"Circulation."},{"key":"B9","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1097\/CRD.0000000000000203","article-title":"Emerging implications of genetic testing in inherited primary arrhythmia syndromes","volume":"27","author":"Asatryan","year":"2019","journal-title":"Cardiol Rev."},{"key":"B10","doi-asserted-by":"publisher","first-page":"2345","DOI":"10.1161\/CIRCULATIONAHA.118.033943","article-title":"Determination and interpretation of the QT interval","volume":"138","author":"Vink","year":"2018","journal-title":"Circulation."},{"key":"B11","doi-asserted-by":"publisher","first-page":"2237","DOI":"10.1161\/01.CIR.97.22.2237","article-title":"Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry","volume":"97","author":"Locati","year":"1998","journal-title":"Circulation."},{"key":"B12","doi-asserted-by":"publisher","first-page":"507","DOI":"10.1016\/j.hrthm.2005.01.020","article-title":"Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing","volume":"2","author":"Tester","year":"2005","journal-title":"Heart Rhythm."},{"key":"B13","doi-asserted-by":"publisher","first-page":"e12537","DOI":"10.1111\/anec.12537","article-title":"Clinical aspects of the three major genetic forms of long QT syndrome (LQT1, LQT2, LQT3)","volume":"23","author":"Kutyifa","year":"2018","journal-title":"Ann Noninvasive Electrocardiol."},{"key":"B14","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1016\/S0735-1097(96)00454-8","article-title":"Age-gender influence on the rate-corrected QT interval and the QT-heart rate relation in families with genotypically characterized long QT syndrome","volume":"29","author":"Lehmann","year":"1997","journal-title":"J Am Coll Cardiol."},{"key":"B15","doi-asserted-by":"publisher","first-page":"H640","DOI":"10.1152\/ajpheart.00864.2013","article-title":"Sex differences in the mechanisms underlying long QT syndrome","volume":"307","author":"Salama","year":"2014","journal-title":"Am J Physiol Heart Circ Physiol."},{"key":"B16","doi-asserted-by":"publisher","first-page":"1866","DOI":"10.1056\/NEJMoa022147","article-title":"Risk stratification in the long-QT syndrome","volume":"348","author":"Priori","year":"2003","journal-title":"N Engl J Med."},{"key":"B17","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1016\/S0735-1097(03)00554-0","article-title":"Modulating effects of age and gender on the clinical course of long QT syndrome by genotype","volume":"42","author":"Zareba","year":"2003","journal-title":"J Am Coll Cardiol."},{"key":"B18","doi-asserted-by":"publisher","first-page":"2784","DOI":"10.1161\/CIRCULATIONAHA.110.000620","article-title":"Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2","volume":"123","author":"Buber","year":"2011","journal-title":"Circulation."},{"key":"B19","doi-asserted-by":"publisher","first-page":"872","DOI":"10.1161\/CIRCULATIONAHA.116.021823","article-title":"Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study","volume":"134","author":"Wilde","year":"2016","journal-title":"Circulation."},{"key":"B20","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1111\/j.1365-2796.2005.01589.x","article-title":"Long QT syndrome: reduced repolarization reserve and the genetic link","volume":"259","author":"Roden","year":"2006","journal-title":"J Intern Med."},{"key":"B21","doi-asserted-by":"publisher","first-page":"2459","DOI":"10.1113\/JP270526","article-title":"Predicting drug-induced QT prolongation and torsades de pointes","volume":"594","author":"Roden","year":"2016","journal-title":"J Physiol."},{"key":"B22","doi-asserted-by":"publisher","first-page":"2590","DOI":"10.1001\/jama.1993.03510210076031","article-title":"Female gender as a risk factor for torsades de pointes associated with cardiovascular drugs","volume":"270","author":"Makkar","year":"1993","journal-title":"JAMA."},{"key":"B23","doi-asserted-by":"publisher","first-page":"282","DOI":"10.1097\/01.md.0000085057.63483.9b","article-title":"Torsade de pointes due to noncardiac drugs: most patients have easily identifiable risk factors","volume":"82","author":"Zeltser","year":"2003","journal-title":"Medicine (Baltimore)."},{"key":"B24","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1016\/j.hrthm.2016.09.013","article-title":"Female gender as independent risk factor of torsades de pointes during acquired atrioventricular block","volume":"14","author":"Chorin","year":"2017","journal-title":"Heart Rhythm."},{"key":"B25","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1111\/bcp.12201","article-title":"Are women more susceptible than men to drug-induced QT prolongation? Concentration-QTc modelling in a phase 1 study with oral rac-sotalol","volume":"77","author":"Darpo","year":"2014","journal-title":"Br J Clin Pharmacol."},{"key":"B26","doi-asserted-by":"publisher","first-page":"1932","DOI":"10.1016\/j.hrthm.2013.05.014","article-title":"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","volume":"10","author":"Priori","year":"2013","journal-title":"Heart Rhythm."},{"key":"B27","doi-asserted-by":"publisher","first-page":"770","DOI":"10.1016\/S0008-6363(01)00477-1","article-title":"Gender differences in the long QT syndrome: effects of beta-adrenoceptor blockade","volume":"53","author":"Conrath","year":"2002","journal-title":"Cardiovasc Res."},{"key":"B28","doi-asserted-by":"publisher","first-page":"2184","DOI":"10.1161\/CIRCULATIONAHA.107.701243","article-title":"Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome","volume":"117","author":"Goldenberg","year":"2008","journal-title":"Circulation."},{"key":"B29","doi-asserted-by":"publisher","first-page":"1147","DOI":"10.1016\/j.hrthm.2020.02.017","article-title":"Intentional nontherapy in long QT syndrome","volume":"17","author":"Macintyre","year":"2020","journal-title":"Heart Rhythm."},{"key":"B30","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1111\/j.1540-8183.1995.tb00930.x","article-title":"Left cardiac sympathetic denervation in long QT syndrome patients","volume":"8","author":"Ferrari","year":"1995","journal-title":"J Interv Cardiol."},{"key":"B31","doi-asserted-by":"publisher","first-page":"3381","DOI":"10.1161\/01.CIR.92.12.3381","article-title":"Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-specific therapy","volume":"92","author":"Schwartz","year":"1995","journal-title":"Circulation."},{"key":"B32","doi-asserted-by":"publisher","first-page":"1053","DOI":"10.1016\/j.jacc.2015.12.033","article-title":"Gene-specific therapy with mexiletine reduces arrhythmic events in patients with long QT syndrome type 3","volume":"67","author":"Mazzanti","year":"2016","journal-title":"J Am Coll Cardiol."},{"key":"B33","doi-asserted-by":"publisher","first-page":"370","DOI":"10.1093\/europace\/euw439","article-title":"Long-term flecainide therapy in type 3 long QT syndrome","volume":"20","author":"Chorin","year":"2018","journal-title":"Europace."},{"key":"B34","doi-asserted-by":"publisher","first-page":"1143","DOI":"10.1016\/j.hrthm.2012.02.006","article-title":"Potential depot medroxyprogesterone acetate-triggered torsades de pointes in a case of congenital type 2 long QT syndrome","volume":"9","author":"Giudicessi","year":"2012","journal-title":"Heart Rhythm."},{"key":"B35","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/j.hrthm.2021.07.058","article-title":"Use of oral contraceptives in women with congenital long QT syndrome","volume":"19","author":"Goldenberg","year":"2022","journal-title":"Heart Rhythm."},{"key":"B36","doi-asserted-by":"publisher","first-page":"2132","DOI":"10.1161\/01.CIR.0000033596.21845.D8","article-title":"Testosterone diminishes the proarrhythmic effects of dofetilide in normal female rabbits","volume":"106","author":"Pham","year":"2002","journal-title":"Circulation."},{"key":"B37","doi-asserted-by":"publisher","first-page":"1070","DOI":"10.1161\/CIRCULATIONAHA.119.040162","article-title":"Androgenic effects on ventricular repolarization: a translational study from the international pharmacovigilance database to iPSC-cardiomyocytes","volume":"140","author":"Salem","year":"2019","journal-title":"Circulation."},{"key":"B38","doi-asserted-by":"publisher","first-page":"1127","DOI":"10.1161\/CIRCULATIONAHA.119.041395","article-title":"Effect of transdermal testosterone and oral progesterone on drug-induced qt interval lengthening in older men: a randomized, double-blind, placebo-controlled crossover-design study","volume":"140","author":"Muensterman","year":"2019","journal-title":"Circulation."},{"key":"B39","doi-asserted-by":"publisher","first-page":"1092","DOI":"10.1016\/j.jacc.2006.09.054","article-title":"Long QT syndrome and pregnancy","volume":"49","author":"Seth","year":"2007","journal-title":"J Am Coll Cardiol."},{"key":"B40","doi-asserted-by":"publisher","first-page":"3165","DOI":"10.1093\/eurheartj\/ehy340","article-title":"2018. ESC Guidelines for the management of cardiovascular diseases during pregnancy","volume":"39","author":"Regitz-Zagrosek","year":"2018","journal-title":"Eur Heart J."},{"key":"B41","doi-asserted-by":"publisher","first-page":"1374","DOI":"10.1136\/heartjnl-2016-310617","article-title":"Arrhythmia risk and beta-blocker therapy in pregnant women with long QT syndrome","volume":"103","author":"Ishibashi","year":"2017","journal-title":"Heart."},{"key":"B42","doi-asserted-by":"publisher","first-page":"e295","DOI":"10.1016\/j.hrthm.2016.05.024","article-title":"J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge","volume":"13","author":"Antzelevitch","year":"2016","journal-title":"Heart Rhythm."},{"key":"B43","doi-asserted-by":"publisher","first-page":"1195","DOI":"10.1161\/CIRCULATIONAHA.118.035070","article-title":"Reappraisal of reported genes for sudden arrhythmic death: evidence-based evaluation of gene validity for Brugada Syndrome","volume":"138","author":"Hosseini","year":"2018","journal-title":"Circulation."},{"key":"B44","doi-asserted-by":"publisher","first-page":"1410","DOI":"10.1016\/j.jacc.2012.04.037","article-title":"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","volume":"60","author":"Crotti","year":"2012","journal-title":"J Am Coll Cardiol."},{"key":"B45","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1111\/j.1540-8167.2006.00743.x","article-title":"Sex hormone and gender difference\u2013role of testosterone on male predominance in Brugada syndrome","volume":"18","author":"Shimizu","year":"2007","journal-title":"J Cardiovasc Electrophysiol."},{"key":"B46","doi-asserted-by":"publisher","first-page":"e005222","DOI":"10.1161\/CIRCEP.117.005222","article-title":"Age of first arrhythmic event in brugada syndrome: data from the SABRUS (Survey on Arrhythmic Events in Brugada Syndrome) in 678 Patients","volume":"10","author":"Milman","year":"2017","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B47","doi-asserted-by":"publisher","first-page":"260","DOI":"10.1016\/j.hrthm.2018.08.032","article-title":"Clinical presentation and follow-up of women affected by Brugada syndrome","volume":"16","author":"Berthome","year":"2019","journal-title":"Heart Rhythm."},{"key":"B48","doi-asserted-by":"publisher","first-page":"1457","DOI":"10.1016\/j.hrthm.2018.06.019","article-title":"Gender differences in patients with Brugada syndrome and arrhythmic events: Data from a survey on arrhythmic events in 678 patients","volume":"15","author":"Milman","year":"2018","journal-title":"Heart Rhythm."},{"key":"B49","doi-asserted-by":"publisher","first-page":"1567","DOI":"10.1016\/j.jacc.2008.07.052","article-title":"Gender differences in clinical manifestations of Brugada syndrome","volume":"52","author":"Benito","year":"2008","journal-title":"J Am Coll Cardiol."},{"key":"B50","doi-asserted-by":"publisher","first-page":"1551","DOI":"10.1046\/j.1460-9592.2003.t01-1-00227.x","article-title":"Disappearance of the Brugada-type electrocardiogram after surgical castration: a role for testosterone and an explanation for the male preponderance","volume":"26","author":"Matsuo","year":"2003","journal-title":"Pacing Clin Electrophysiol."},{"key":"B51","doi-asserted-by":"publisher","first-page":"2448","DOI":"10.1253\/circj.CJ-10-0221","article-title":"Gender differences in the ST segment: effect of androgen-deprivation therapy and possible role of testosterone","volume":"74","author":"Ezaki","year":"2010","journal-title":"Circ J."},{"key":"B52","doi-asserted-by":"publisher","first-page":"284","DOI":"10.1046\/j.1542-474X.2003.08404.x","article-title":"Comparison of electrocardiographic repolarization patterns between hypogonad males and normal subjects","volume":"8","author":"Kirilmaz","year":"2003","journal-title":"Ann Noninvasive Electrocardiol."},{"key":"B53","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1253\/circj.CJ-08-0680","article-title":"Men with Brugada-like electrocardiogram have higher risk of prostate cancer","volume":"73","author":"Haruta","year":"2009","journal-title":"Circ J."},{"key":"B54","doi-asserted-by":"publisher","first-page":"452","DOI":"10.1136\/heartjnl-2015-308556","article-title":"Clinical characterisation and long-term prognosis of women with Brugada syndrome","volume":"102","author":"Sieira","year":"2016","journal-title":"Heart"},{"key":"B55","doi-asserted-by":"publisher","first-page":"3081","DOI":"10.1161\/hc5001.100834","article-title":"Novel SCN5A mutation leading either to isolated cardiac conduction defect or Brugada syndrome in a large French family","volume":"104","author":"Kyndt","year":"2001","journal-title":"Circulation."},{"key":"B56","doi-asserted-by":"publisher","first-page":"e009387","DOI":"10.1161\/JAHA.118.009387","article-title":"Sex-dependent phenotypic variability of an SCN5A mutation: brugada syndrome and sick sinus syndrome","volume":"7","author":"Aizawa","year":"2018","journal-title":"J Am Heart Assoc."},{"key":"B57","doi-asserted-by":"publisher","first-page":"E101","DOI":"10.1093\/eurheartj\/suaa071","article-title":"Right ventricular electromechanical abnormalities in Brugada syndrome: is this a cardiomyopathy?","volume":"22","author":"Pappone","year":"2020","journal-title":"Eur Heart J Suppl."},{"key":"B58","doi-asserted-by":"publisher","first-page":"e005053","DOI":"10.1161\/CIRCEP.117.005053","article-title":"Electrical substrate elimination in 135 consecutive patients with Brugada syndrome","volume":"10","author":"Pappone","year":"2017","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B59","doi-asserted-by":"publisher","first-page":"1082","DOI":"10.1093\/eurheartj\/ehaa942","article-title":"Brugada syndrome genetics is associated with phenotype severity","volume":"42","author":"Ciconte","year":"2021","journal-title":"Eur Heart J."},{"key":"B60","doi-asserted-by":"publisher","first-page":"1739","DOI":"10.1161\/CIRCULATIONAHA.113.001941","article-title":"Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study-part 2","volume":"128","author":"Sacher","year":"2013","journal-title":"Circulation."},{"key":"B61","doi-asserted-by":"publisher","first-page":"1393","DOI":"10.1161\/CIRCEP.115.003109","article-title":"Management of Brugada syndrome: thirty-three-year experience using electrophysiologically guided therapy with class 1A antiarrhythmic drugs","volume":"8","author":"Belhassen","year":"2015","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B62","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1016\/j.jacep.2018.10.007","article-title":"Challenge and impact of quinidine access in sudden death syndromes: a national experience","volume":"5","author":"Malhi","year":"2019","journal-title":"JACC Clin Electrophysiol."},{"key":"B63","doi-asserted-by":"publisher","first-page":"1270","DOI":"10.1161\/CIRCULATIONAHA.110.972612","article-title":"Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium","volume":"123","author":"Nademanee","year":"2011","journal-title":"Circulation."},{"key":"B64","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1016\/j.rec.2013.06.023","article-title":"The clinical significance of pregnancy in Brugada syndrome","volume":"67","author":"Rodriguez-Manero","year":"2014","journal-title":"Rev Esp Cardiol (Engl Ed)."},{"key":"B65","doi-asserted-by":"publisher","first-page":"675","DOI":"10.1016\/j.hrthm.2006.12.048","article-title":"Diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia","volume":"4","author":"Napolitano","year":"2007","journal-title":"Heart Rhythm."},{"key":"B66","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1161\/CIRCEP.114.002217","article-title":"Catecholaminergic polymorphic ventricular tachycardia in children: analysis of therapeutic strategies and outcomes from an international multicenter registry","volume":"8","author":"Roston","year":"2015","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B67","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1161\/01.CIR.0000020013.73106.D8","article-title":"Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia","volume":"106","author":"Priori","year":"2002","journal-title":"Circulation."},{"key":"B68","doi-asserted-by":"publisher","first-page":"S96","DOI":"10.1016\/j.cjca.2017.07.191","article-title":"Sex-influenced mortality in three well-ascertained families with catecholaminergic polymorphic ventricular tachycardia caused by a RYR2 p.R420W mutation: the power of extended family history","volume":"33","author":"Abdel-Razek","year":"2017","journal-title":"Can J Cardiol."},{"key":"B69","doi-asserted-by":"publisher","first-page":"1308","DOI":"10.1016\/j.jacep.2017.05.004","article-title":"Circadian variation of ventricular arrhythmias in catecholaminergic polymorphic ventricular tachycardia","volume":"3","author":"Miyake","year":"2017","journal-title":"JACC Clin Electrophysiol."},{"key":"B70","doi-asserted-by":"publisher","first-page":"759","DOI":"10.1001\/jamacardio.2017.1320","article-title":"Efficacy of flecainide in the treatment of catecholaminergic polymorphic ventricular tachycardia: a randomized clinical trial","volume":"2","author":"Kannankeril","year":"2017","journal-title":"JAMA Cardiol."},{"key":"B71","doi-asserted-by":"publisher","first-page":"1791","DOI":"10.1016\/j.hrthm.2018.06.046","article-title":"Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review","volume":"15","author":"Roston","year":"2018","journal-title":"Heart Rhythm."},{"key":"B72","doi-asserted-by":"publisher","first-page":"2953","DOI":"10.1093\/eurheartj\/ehz309","article-title":"Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest","volume":"40","author":"Van Der Werf","year":"2019","journal-title":"Eur Heart J."},{"key":"B73","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1016\/j.jacep.2018.10.019","article-title":"Pregnancy in catecholaminergic polymorphic ventricular tachycardia","volume":"5","author":"Cheung","year":"2019","journal-title":"JACC Clin Electrophysiol."},{"key":"B74","doi-asserted-by":"publisher","first-page":"2793","DOI":"10.1093\/eurheartj\/ehv316","article-title":"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)","volume":"36","author":"Priori","year":"2015","journal-title":"Eur Heart J."},{"key":"B75","doi-asserted-by":"publisher","first-page":"1300","DOI":"10.1016\/j.jacc.2013.09.078","article-title":"Novel insight into the natural history of short QT syndrome","volume":"63","author":"Mazzanti","year":"2014","journal-title":"J Am Coll Cardiol."},{"key":"B76","doi-asserted-by":"publisher","first-page":"1335","DOI":"10.1177\/2047487319850953","article-title":"Sex-differences in short QT syndrome: a systematic literature review and pooled analysis","volume":"27","author":"El-Battrawy","year":"2020","journal-title":"Eur J Prev Cardiol."},{"key":"B77","doi-asserted-by":"publisher","first-page":"3010","DOI":"10.1016\/j.jacc.2017.10.025","article-title":"Hydroquinidine prevents life-threatening arrhythmic events in patients with short QT syndrome","volume":"70","author":"Mazzanti","year":"2017","journal-title":"J Am Coll Cardiol."},{"key":"B78","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1002\/clc.4960220203","article-title":"Early repolarization","volume":"22","author":"Mehta","year":"1999","journal-title":"Clin Cardiol."},{"key":"B79","doi-asserted-by":"publisher","first-page":"392","DOI":"10.1161\/CIRCEP.113.000874","article-title":"Clinical characteristics and outcomes associated with the natural history of early repolarization in a young, biracial cohort followed to middle age: the Coronary Artery Risk Development in Young Adults (CARDIA) study","volume":"7","author":"Ilkhanoff","year":"2014","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B80","doi-asserted-by":"publisher","first-page":"2016","DOI":"10.1056\/NEJMoa071968","article-title":"Sudden cardiac arrest associated with early repolarization","volume":"358","author":"Haissaguerre","year":"2008","journal-title":"N Engl J Med."},{"key":"B81","doi-asserted-by":"publisher","first-page":"2284","DOI":"10.1016\/S0735-1097(11)60109-5","article-title":"The early repolarization pattern in the general population: clinical correlates and heritability","volume":"57","author":"Noseworthy","year":"2011","journal-title":"J Am Coll Cardiol."},{"key":"B82","doi-asserted-by":"publisher","first-page":"432","DOI":"10.1161\/CIRCEP.111.962852","article-title":"Early repolarization pattern in competitive athletes: clinical correlates and the effects of exercise training","volume":"4","author":"Noseworthy","year":"2011","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B83","doi-asserted-by":"publisher","first-page":"549","DOI":"10.1016\/j.hrthm.2009.12.006","article-title":"J wave syndromes","volume":"7","author":"Antzelevitch","year":"2010","journal-title":"Heart Rhythm."},{"key":"B84","doi-asserted-by":"publisher","first-page":"2666","DOI":"10.1161\/CIRCULATIONAHA.110.014068","article-title":"Early repolarization: electrocardiographic phenotypes associated with favorable long-term outcome","volume":"123","author":"Tikkanen","year":"2011","journal-title":"Circulation."},{"key":"B85","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1093\/europace\/euw394","article-title":"A meta-analysis on the prognostic significance of inferolateral early repolarization pattern in Brugada syndrome","volume":"20","author":"Georgopoulos","year":"2018","journal-title":"Europace."},{"key":"B86","doi-asserted-by":"publisher","first-page":"647","DOI":"10.1016\/j.hrthm.2010.01.012","article-title":"High prevalence of early repolarization in short QT syndrome","volume":"7","author":"Watanabe","year":"2010","journal-title":"Heart Rhythm."},{"key":"B87","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.1093\/europace\/euv357","article-title":"Early repolarization pattern: a marker of increased risk in patients with catecholaminergic polymorphic ventricular tachycardia","volume":"18","author":"Tulumen","year":"2016","journal-title":"Europace."},{"key":"B88","doi-asserted-by":"publisher","first-page":"1632","DOI":"10.1016\/j.hrthm.2014.05.027","article-title":"Early repolarization is associated with symptoms in patients with type 1 and type 2 long QT syndrome","volume":"11","author":"Laksman","year":"2014","journal-title":"Heart Rhythm."},{"key":"B89","doi-asserted-by":"publisher","first-page":"1238","DOI":"10.1016\/j.jacep.2018.06.007","article-title":"Clinical significance of early repolarization in long QT syndrome","volume":"4","author":"Sugrue","year":"2018","journal-title":"JACC Clin Electrophysiol."},{"key":"B90","doi-asserted-by":"publisher","first-page":"266","DOI":"10.3389\/fphys.2016.00266","article-title":"Early repolarization syndrome; mechanistic theories and clinical correlates","volume":"7","author":"Mercer","year":"2016","journal-title":"Front Physiol."},{"key":"B91","doi-asserted-by":"publisher","first-page":"1633","DOI":"10.1016\/j.jacc.2013.07.015","article-title":"Relationship between testosterone level and early repolarization on 12-lead electrocardiograms in men","volume":"62","author":"Junttila","year":"2013","journal-title":"J Am Coll Cardiol."},{"key":"B92","doi-asserted-by":"publisher","first-page":"1473","DOI":"10.1016\/j.jacep.2018.07.001","article-title":"Early Repolarization Pattern Inheritance in the Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER)","volume":"4","author":"Malhi","year":"2018","journal-title":"JACC Clin Electrophysiol."},{"key":"B93","doi-asserted-by":"publisher","first-page":"1981","DOI":"10.1093\/eurheartj\/ehy098","article-title":"Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation","volume":"39","author":"Waldmann","year":"2018","journal-title":"Eur Heart J."},{"key":"B94","doi-asserted-by":"publisher","first-page":"278","DOI":"10.1161\/CIRCULATIONAHA.109.853143","article-title":"Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER)","volume":"120","author":"Krahn","year":"2009","journal-title":"Circulation."},{"key":"B95","doi-asserted-by":"publisher","first-page":"661","DOI":"10.1016\/0002-8703(90)90025-S","article-title":"Idiopathic ventricular fibrillation","volume":"120","author":"Viskin","year":"1990","journal-title":"Am Heart J."},{"key":"B96","doi-asserted-by":"publisher","first-page":"962","DOI":"10.1161\/01.CIR.0000027564.55739.B1","article-title":"Mapping and ablation of idiopathic ventricular fibrillation","volume":"106","author":"Haissaguerre","year":"2002","journal-title":"Circulation."},{"key":"B97","doi-asserted-by":"publisher","first-page":"1288","DOI":"10.1016\/j.jacc.2005.05.077","article-title":"Malignant entity of idiopathic ventricular fibrillation and polymorphic ventricular tachycardia initiated by premature extrasystoles originating from the right ventricular outflow tract","volume":"46","author":"Noda","year":"2005","journal-title":"J Am Coll Cardiol."},{"key":"B98","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1161\/CIRCGENETICS.116.001686","article-title":"Genetic testing in the evaluation of unexplained cardiac arrest: from the CASPER (Cardiac Arrest Survivors With Preserved Ejection Fraction Registry)","author":"Mellor","year":"2017","journal-title":"Circ Cardiovasc Genet."},{"key":"B99","doi-asserted-by":"publisher","first-page":"2031","DOI":"10.1016\/j.amjcard.2019.02.061","article-title":"Usefulness of genetic testing in sudden cardiac arrest survivors with or without previous clinical evidence of heart disease","volume":"123","author":"Asatryan","year":"2019","journal-title":"Am J Cardiol."},{"key":"B100","doi-asserted-by":"publisher","first-page":"2964","DOI":"10.1093\/eurheartj\/ehz311","article-title":"Calmodulin mutations and life-threatening cardiac arrhythmias: insights from the International Calmodulinopathy Registry","volume":"40","author":"Crotti","year":"2019","journal-title":"Eur Heart J."},{"key":"B101","doi-asserted-by":"publisher","first-page":"1095","DOI":"10.1111\/jce.12737","article-title":"Long-term outcome of patients with idiopathic ventricular fibrillation: a meta-analysis","volume":"26","author":"Ozaydin","year":"2015","journal-title":"J Cardiovasc Electrophysiol."},{"key":"B102","doi-asserted-by":"publisher","first-page":"e003619","DOI":"10.1161\/CIRCEP.115.003619","article-title":"Outcome of apparently unexplained cardiac arrest: results from investigation and follow-up of the prospective cardiac arrest survivors with preserved ejection fraction registry","volume":"9","author":"Herman","year":"2016","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B103","doi-asserted-by":"publisher","first-page":"e004258","DOI":"10.1161\/CIRCEP.116.004258","article-title":"Long-term outcome of patients initially diagnosed with idiopathic ventricular fibrillation: a descriptive study","volume":"9","author":"Visser","year":"2016","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B104","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1016\/j.hrthm.2015.12.006","article-title":"Detailed characterization of familial idiopathic ventricular fibrillation linked to the DPP6 locus","volume":"13","author":"Ten Sande","year":"2016","journal-title":"Heart Rhythm."},{"key":"B105","doi-asserted-by":"publisher","first-page":"123","DOI":"10.3389\/fcvm.2018.00123","article-title":"Mapping and ablation of idiopathic ventricular fibrillation","volume":"5","author":"Cheniti","year":"2018","journal-title":"Front Cardiovasc Med."},{"key":"B106","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1161\/CIRCEP.115.004274","article-title":"Cardiac abnormalities in first-degree relatives of unexplained cardiac arrest victims: a report from the cardiac arrest survivors with preserved ejection fraction registry","author":"Steinberg","year":"2016","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B107","doi-asserted-by":"publisher","first-page":"1465","DOI":"10.1001\/jama.225.12.1465","article-title":"Hereditary progressive atrioventricular conduction defect: a new syndrome?","volume":"225","author":"Lynch","year":"1973","journal-title":"JAMA."},{"key":"B108","doi-asserted-by":"publisher","first-page":"1145","DOI":"10.1093\/europace\/euz109","article-title":"Molecular and genetic insights into progressive cardiac conduction disease","volume":"21","author":"Asatryan","year":"2019","journal-title":"Europace."},{"key":"B109","doi-asserted-by":"publisher","first-page":"1780","DOI":"10.1056\/NEJMc1807668","article-title":"A novel familial cardiac arrhythmia syndrome with widespread ST-segment depression","volume":"379","author":"Bundgaard","year":"2018","journal-title":"N Engl J Med"},{"key":"B110","doi-asserted-by":"publisher","first-page":"e010688","DOI":"10.1161\/CIRCEP.121.010688","article-title":"Electrocardiographic findings, arrhythmias, and left ventricular involvement in familial ST-depression syndrome","volume":"15","author":"Christensen","year":"2022","journal-title":"Circ Arrhythm Electrophysiol."},{"key":"B111","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1097\/00000542-200501000-00029","article-title":"Anesthesia for patients with congenital long QT syndrome","volume":"102","author":"Kies","year":"2005","journal-title":"Anesthesiology."},{"key":"B112","doi-asserted-by":"publisher","first-page":"404","DOI":"10.1038\/ejhg.2016.199","article-title":"Post-mortem whole-exome analysis in a large sudden infant death syndrome cohort with a focus on cardiovascular and metabolic genetic diseases","volume":"25","author":"Neubauer","year":"2017","journal-title":"Eur J Hum Genet."},{"key":"B113","doi-asserted-by":"publisher","first-page":"1516","DOI":"10.1111\/pace.12246","article-title":"Safe single-dose administration of propofol in patients with established Brugada syndrome: a retrospective database analysis","volume":"36","author":"Flamee","year":"2013","journal-title":"Pacing Clin Electrophysiol."},{"key":"B114","doi-asserted-by":"publisher","first-page":"1126","DOI":"10.1093\/europace\/euz037","article-title":"Postpartum hormones oxytocin and prolactin cause pro-arrhythmic prolongation of cardiac repolarization in long QT syndrome type 2","volume":"21","author":"Bodi","year":"2019","journal-title":"Europace."}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2022.1010748\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,1,4]],"date-time":"2023-01-04T13:45:53Z","timestamp":1672839953000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2022.1010748\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,4]]},"references-count":114,"alternative-id":["10.3389\/fcvm.2022.1010748"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2022.1010748","relation":{},"ISSN":["2297-055X"],"issn-type":[{"value":"2297-055X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,4]]},"article-number":"1010748"}}