Mechanisms and clinical management of inherited channelopathies: long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, and short QT syndrome

Brugada Syndrome: Report of the Second Consensus Conference

Antzelevitch, C. N., Brugada, P., Borggrefe, M. M., Brugada, J., Brugada, R., Corrado, D., Gussak, I. B., Marec, H. L., Nademanee, K., Pérez‐Riera, A. R., et al. (2005) · Circulation · doi:10.1161/01.cir.0000152479.54298.51 · Cited by 1,807 · see citing works

Genotype-Phenotype Correlation in the Long-QT Syndrome: Gene-Specific Triggers for Life-Threatening Arrhythmias

Schwartz, P. J., Priori, S. G., Spazzolini, C., Moss, A. J., Vincent, G. M., Napolitano, C., Denjoy, I., Guicheney, P., Breithardt, G., Keating, M. T., et al. (2001) · Circulation · doi:10.1161/01.cir.103.1.89 · Cited by 1,779 · see citing works

Risk Stratification in the Long-QT Syndrome

Priori, S. G., Schwartz, P. J., Napolitano, C., Bloise, R., Ronchetti, E., Grillo, M., Vicentini, A., Spazzolini, C., Nastoli, J., Bottelli, G., et al. (2003) · New England Journal of Medicine · doi:10.1056/nejmoa022147 · Cited by 1,456 · see citing works

Natural History of Brugada Syndrome

Priori, S. G., Napolitano, C., Gasparini, M., Pappone, C., Bella, P. E. D., Giordano, U., Bloise, R., Giustetto, C., Nardis, R. D., Grillo, M., et al. (2002) · Circulation · doi:10.1161/hc1102.105288 · Cited by 1,030 · see citing works

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

Antzelevitch, C., Pollevick, G. D., Cordeiro, J. M., Casis, O., Sanguinetti, M. C., Aizawa, Y., Guerchicoff, A., Pfeiffer, R., Oliva, A., Wollnik, B., et al. (2007) · Circulation · doi:10.1161/circulationaha.106.668392 · Cited by 933 · see citing works

Sudden death associated with short-QT syndrome linked to mutations in HERG

Brugada, R., Hong, K., Dumaine, R., Cordeiro, J., Gaita, F., Borggrefe, M., Menendez, T. M., Brugada, J., Pollevick, G. D., Wolpert, C., et al. (2004) · Circulation · doi:10.1161/01.cir.0000109482.92774.3a · Cited by 847 · see citing works

Effectiveness and Limitations of β-Blocker Therapy in Congenital Long-QT Syndrome

Moss, A. J., Zaręba, W., Hall, W. J., Schwartz, P. J., Crampton, R. S., Benhorin, J., Vincent, G. M., Locati, E. H., Priori, S. G., Napolitano, C., et al. (2000) · Circulation · doi:10.1161/01.cir.101.6.616 · Cited by 836 · see citing works

Low Penetrance in the Long-QT Syndrome

Priori, S. G., Napolitano, C., & Schwartz, P. J. (1999) · Circulation · doi:10.1161/01.cir.99.4.529 · Cited by 814 · see citing works

Short QT Syndrome: A Familial Cause of Sudden Death

Gaita, F., Giustetto, C., Bianchi, F., Wolpert, C., Schimpf, R., Riccardi, R., Grossi, S., Richiardi, E., & Borggrefe, M. (2003) · Circulation · doi:10.1161/01.cir.0000085071.28695.c4 · Cited by 708 · see citing works

Left Cardiac Sympathetic Denervation in the Management of High-Risk Patients Affected by the Long-QT Syndrome

Schwartz, P. J., Priori, S. G., Cerrone, M., Spazzolini, C., Odero, A., Napolitano, C., Bloise, R., Ferrari, G. M. D., Klersy, C., Moss, A. J., et al. (2004) · Circulation · doi:10.1161/01.cir.0000125523.14403.1e · Cited by 679 · see citing works

Idiopathic Short QT Interval:A New Clinical Syndrome?

Gussak, I. B., Brugada, P., Brugada, J., Wright, R. S., Kopecky, S. L., CHAITMAN, B. R., & Bjerregaard, P. (2000) · Cardiology · doi:10.1159/000047299 · Cited by 669 · see citing works

A Novel Form of Short QT Syndrome (SQT3) Is Caused by a Mutation in the KCNJ2 Gene

Priori, S. G., Pandit, S. V., Rivolta, I., Berenfeld, O., Ronchetti, E., Dhamoon, A. S., Napolitano, C., ANUMONWO, J. M. B., di Barletta, M. R., Gudapakkam, S., et al. (2005) · Circulation Research · doi:10.1161/01.res.0000162101.76263.8c · Cited by 624 · see citing works

The Spectrum of Symptoms and QT Intervals in Carriers of the Gene for the Long-QT Syndrome

Vincent, G. M., Timothy, K. W., Leppert, M., & Keating, M. T. (1992) · New England Journal of Medicine · doi:10.1056/nejm199209173271204 · Cited by 621 · see citing works

ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome

Moss, A. J., Zareba, W., Benhorin, J., Locati, E. H., Hall, W. J., Robinson, J. L., Schwartz, P. J., Towbin, J. A., Vincent, G. M., Lehmann, M. H., et al. (1995) · Circulation · doi:10.1161/01.cir.92.10.2929 · Cited by 616 · see citing works

Mutation in the KCNQ1 Gene Leading to the Short QT-Interval Syndrome

Bellocq, C., van Ginneken, A. C. G., Bezzina, C. R., Alders, M., Escande, D., Mannens, M. M. A. M., Baró, I., & Wilde, A. A. M. (2004) · Circulation · doi:10.1161/01.cir.0000130409.72142.fe · Cited by 614 · see citing works

Association of Long QT Syndrome Loci and Cardiac Events Among Patients Treated With β-Blockers

Priori, S. G. (2004) · JAMA · doi:10.1001/jama.292.11.1341 · Cited by 592 · see citing works

Prevalence of Long-QT Syndrome Gene Variants in Sudden Infant Death Syndrome

Arnestad, M., Crotti, L., Rognum, T. O., Insolia, R., Pedrazzini, M., Ferrandi, C., Vege, Å., Wang, D. W., Rhodes, T. E., George, A. L., et al. (2007) · Circulation · doi:10.1161/circulationaha.106.658021 · Cited by 548 · see citing works

Determinants of Sudden Cardiac Death in Individuals With the Electrocardiographic Pattern of Brugada Syndrome and No Previous Cardiac Arrest

Brugada, J., Brugada, R., & Brugada, P. (2003) · Circulation · doi:10.1161/01.cir.0000104568.13957.4f · Cited by 546 · see citing works

Sodium channel β1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans.

Watanabe, H., Koopmann, T., Scouarnec, S. L., Yang, T., Ingram, C. R., Schott, J., Demolombe, S., Probst, V., Anselme, F., Escande, D., et al. (2008) · Journal of Clinical Investigation · doi:10.1172/jci33891 · Cited by 507 · see citing works

The Brugada syndrome: clinical, electrophysiologic and genetic aspects

Gussak, I. B., Antzelevitch, C. N., Bjerregaard, P., Towbin, J., & CHAITMAN, B. R. (1999) · Journal of the American College of Cardiology · doi:10.1016/s0735-1097(98)00528-2 · Cited by 501 · see citing works

Clinical Aspects of Type-1 Long-QT Syndrome by Location, Coding Type, and Biophysical Function of Mutations Involving the KCNQ1 Gene

Moss, A. J., Shimizu, W., Wilde, A. A. M., Towbin, J. A., Zaręba, W., Robinson, J. L., Qi, M., Vincent, G. M., Ackerman, M. J., Kaufman, E. S., et al. (2007) · Circulation · doi:10.1161/circulationaha.106.665406 · Cited by 461 · see citing works

Mutation in Glycerol-3-Phosphate Dehydrogenase 1–Like Gene ( <i>GPD1-L</i> ) Decreases Cardiac Na ⁺ Current and Causes Inherited Arrhythmias

London, B., Michalec, M., Mehdi, H., Zhu, X., Kerchner, L., Sanyal, S., Viswanathan, P. C., Pfahnl, A. E., Shang, L. L., Madhusudanan, M., et al. (2007) · Circulation · doi:10.1161/circulationaha.107.703330 · Cited by 437 · see citing works

Left Cardiac Sympathetic Denervation for Catecholaminergic Polymorphic Ventricular Tachycardia

Wilde, A. A. M., Bhuiyan, Z. A., Crotti, L., et al. (2008) · New England Journal of Medicine · doi:10.1056/nejmoa0708006 · Cited by 432 · see citing works

Long QT Syndrome in Adults

Sauer, A. J., Moss, A. J., McNitt, S. A., Peterson, D. R., Zaręba, W., Robinson, J. L., Qi, M., Goldenberg, I., Hobbs, J. B., Ackerman, M. J., et al. (2007) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2006.08.057 · Cited by 417 · see citing works

The Jervell and Lange-Nielsen Syndrome

Schwartz, P. J., Spazzolini, C., Crotti, L., Bathen, J., Amlie, J., Timothy, K. W., Shkolnikova, M. A., Berul, C. I., Bitner-Glindzicz, M. A. K., Toivonen, L. K., et al. (2006) · Circulation · doi:10.1161/circulationaha.105.592899 · Cited by 406 · see citing works

Increased Risk of Arrhythmic Events in Long-QT Syndrome With Mutations in the Pore Region of the Human Ether-a-go-go–Related Gene Potassium Channel

Moss, A. J., Zaręba, W., Kaufman, E. S., Gartman, E. J., Peterson, D. R., Benhorin, J., Towbin, J. A., Keating, M. T., Priori, S. G., Schwartz, P. J., et al. (2002) · Circulation · doi:10.1161/hc0702.105124 · Cited by 396 · see citing works

Short QT syndrome: pharmacological treatment

Gaïta, F., Giustetto, C., Bianchi, F., Schimpf, R., Haı̈ssaguerre, M., Calò, L., Brugada, R., Antzelevitch, C. N., Borggrefe, M. M., & Wolpert, C. (2004) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2004.02.034 · Cited by 358 · see citing works

The long QT syndromes: genetic basis and clinical implications

Chiang, C., & Roden, D. M. (2000) · Journal of the American College of Cardiology · doi:10.1016/s0735-1097(00)00716-6 · Cited by 325 · see citing works

Risk of Aborted Cardiac Arrest or Sudden Cardiac Death During Adolescence in the Long-QT Syndrome

Hobbs, J. B., Peterson, D. R., Moss, A. J., McNitt, S. A., Zaręba, W., Goldenberg, I., Qi, M., Robinson, J. L., Sauer, A. J., Ackerman, M. J., et al. (2006) · JAMA · doi:10.1001/jama.296.10.1249 · Cited by 313 · see citing works

Brugada Syndrome

Antzelevitch, C. (2006) · Pacing and Clinical Electrophysiology · doi:10.1111/j.1540-8159.2006.00507.x · Cited by 303 · see citing works

Implantable Cardioverter Defibrillator in High‐Risk Long QT Syndrome Patients

Zaręba, W., Moss, A. J., Daubert, J. P., Hall, W. J., Robinson, J. L., & Andrews, M. L. (2003) · Journal of Cardiovascular Electrophysiology · doi:10.1046/j.1540-8167.2003.02545.x · Cited by 302 · see citing works

Phase 2 reentry as a mechanism of initiation of circus movement reentry in canine epicardium exposed to simulated ischemia

Lukas, A., & Antzelevitch, C. (1996) · Cardiovascular Research · doi:10.1016/s0008-6363(96)00115-0 · Cited by 218 · see citing works

Cardiac potassium channel dysfunction in sudden infant death syndrome

Rhodes, T. E., Abraham, R. L., Welch, R. C., Vanoye, C. G., Crotti, L., Arnestad, M., Insolia, R., Pedrazzini, M., Ferrandi, C., Vege, Å., et al. (2008) · Journal of Molecular and Cellular Cardiology · doi:10.1016/j.yjmcc.2007.11.015 · Cited by 216 · see citing works

Patients With an Asymptomatic Brugada Electrocardiogram Should Undergo Pharmacological and Electrophysiological Testing

Brugada, P., Brugada, R., & Brugada, J. (2005) · Circulation · doi:10.1161/circulationaha.104.485326 · Cited by 204 · see citing works

Sodium channel beta 1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans

Watanabe, H., Koopmann, T. T., Scouarnec, S. L., Yang, T., Ingram, C. R., Schott, J.-J., Demolombe, S., Probst, V., Anselme, F., Escande, D., et al. (2008) · Pure Amsterdam UMC · doi:10.1172/jc133891 · Cited by 196 · see citing works

Effects of Flecainide in Patients With New SCN5A Mutation

Benhorin, J., Taub, R., Goldmit, M., Kerem, B., Kass, R. S., Windman, I., & Medina, A. (2000) · Circulation · doi:10.1161/01.cir.101.14.1698 · Cited by 195 · see citing works

Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome.

Moss, A. J., Liu, J. E., Gottlieb, S., Locati, E. H., Schwartz, P. J., & Robinson, J. L. (1991) · Circulation · doi:10.1161/01.cir.84.4.1524 · Cited by 193 · see citing works

Cardiac Sodium Channel Dysfunction in Sudden Infant Death Syndrome

Wang, D. W., Desai, R. R., Crotti, L., Arnestad, M., Insolia, R., Pedrazzini, M., Ferrandi, C., Vege, Å., Rognum, T. O., Schwartz, P. J., et al. (2007) · Circulation · doi:10.1161/circulationaha.106.646513 · Cited by 187 · see citing works

A mechanism for sudden infant death syndrome (SIDS): Stress-induced leak via ryanodine receptors

Tester, D. J., Důra, M., Carturan, E., et al. (2007) · Heart Rhythm · doi:10.1016/j.hrthm.2007.02.026 · Cited by 186 · see citing works

Effect of Clinical Phenotype on Yield of Long QT Syndrome Genetic Testing

Tester, D. J., Will, M. L., Haglund, C. M., & Ackerman, M. J. (2006) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2005.09.056 · Cited by 184 · see citing works

Molecular Genetic Insights into Cardiovascular Disease

Keating, M. T., & Sanguinetti, M. C. (1996) · Science · doi:10.1126/science.272.5262.681 · Cited by 183 · see citing works

Congenital Short QT Syndrome and Implantable Cardioverter Defibrillator Treatment:

Schimpf, R., Wolpert, C., BIANCHI, F., Giustetto, C., Gaïta, F., Bauersfeld, U., & Borggrefe, M. (2003) · Journal of Cardiovascular Electrophysiology · doi:10.1046/j.1540-8167.2003.03278.x · Cited by 176 · see citing works

Molecular and Functional Characterization of Novel Glycerol-3-Phosphate Dehydrogenase 1–Like Gene ( GPD1-L ) Mutations in Sudden Infant Death Syndrome

Norstrand, D. W. V., Valdivia, C. R., Tester, D. J., Ueda, K., London, B., Makielski, J. C., & Ackerman, M. J. (2007) · Circulation · doi:10.1161/circulationaha.107.704627 · Cited by 174 · see citing works

The Idiopathic Long QT Syndrome: Pathogenetic Mechanisms and Therapy

Schwartz, P. J., & Locati, E. H. (1985) · European Heart Journal · doi:10.1093/eurheartj/6.suppl_d.103 · Cited by 150 · see citing works

Diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia

Napolitano, C., & Priori, S. G. (2007) · Heart Rhythm · doi:10.1016/j.hrthm.2006.12.048 · Cited by 148 · see citing works

Long-Term Follow-Up of Patients With Long-QT Syndrome Treated With β-Blockers and Continuous Pacing

Dorostkar, P. C., Eldar, M., Belhassen, B., & Scheinman, M. M. (1999) · Circulation · doi:10.1161/01.cir.100.24.2431 · Cited by 144 · see citing works

Safety and Efficacy of Flecainide in Subjects with Long QT‐3 Syndrome (ΔKPQ Mutation): A Randomized, Double‐Blind, Placebo‐Controlled Clinical Trial

Moss, A. J., Windle, J. R., Hall, W. J., Zaręba, W., Robinson, J. L., McNitt, S. A., Severski, P. K., Rosero, S. Z., Daubert, J. P., Qi, M., et al. (2005) · Annals of Noninvasive Electrocardiology · doi:10.1111/j.1542-474x.2005.00077.x · Cited by 143 · see citing works

Inherited Long QT Syndromes:

Roden, D. M., & Spooner, P. M. (1999) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.1999.tb00231.x · Cited by 140 · see citing works

Clinical features of the idiopathic long QT syndrome.

Moss, A. J., & Robinson, J. (1992) · PubMed · Cited by 126 · see citing works

Normalization of Ventricular Repolarization with Flecainide in Long QT Syndrome Patients with SCN5A:ΔKPQ Mutation

Windle, J. R., Geletka, R., Moss, A. J., Zaręba, W., & Atkins, D. L. (2001) · Annals of Noninvasive Electrocardiology · doi:10.1111/j.1542-474x.2001.tb00100.x · Cited by 126 · see citing works

Molecular and Electrophysiological Bases of Catecholaminergic Polymorphic Ventricular Tachycardia

Mohamed, U., Napolitano, C., & Priori, S. G. (2007) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.2007.00766.x · Cited by 118 · see citing works

Arrhythmias in the congenital long QT syndrome: how often is torsade de pointes pause dependent?

Viskin, S., Fish, R., Zeltser, D., Belhassen, B., Heller, K., Brosh, D., Laniado, S., & Barron, H. V. (2000) · Heart · doi:10.1136/heart.83.6.661 · Cited by 99 · see citing works

Reduction of Dispersion of Repolarization and Prolongation of Postrepolarization Refractoriness Explain the Antiarrhythmic Effects of Quinidine in a Model of Short QT Syndrome

Milberg, P., TEGELKAMP, R., Osada, N., Schimpf, R., Wolpert, C., Breithardt, G., Borggrefe, M., & Eckardt, L. (2007) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.2007.00813.x · Cited by 96 · see citing works

Short QT syndrome: Successful prevention of sudden cardiac death in an adolescent by implantable cardioverter-defibrillator treatment for primary prophylaxis

Schimpf, R., Bauersfeld, U., Gaïta, F., & Wolpert, C. (2005) · Heart Rhythm · doi:10.1016/j.hrthm.2004.11.026 · Cited by 90 · see citing works

Long QT Syndrome in Children in the Era of Implantable Defibrillators

Etheridge, S. P., Sanatani, S., Cohen, M. I., et al. (2007) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2007.05.042 · Cited by 89 · see citing works

Near-miss SIDS due to Brugada syndrome

Skinner, J. R. (2005) · Archives of Disease in Childhood · doi:10.1136/adc.2004.058115 · Cited by 84 · see citing works

Repolarization heterogeneity in the right ventricular outflow tract: Correlation with ventricular arrhythmias in Brugada patients and in an in vitro canine Brugada model

Morita, H., Zipes, D. P., Fukushima‐Kusano, K., Nagase, S., Nakamura, K., Morita, S. T., Ohe, T., & Wu, J. (2008) · Heart Rhythm · doi:10.1016/j.hrthm.2008.02.028 · Cited by 73 · see citing works

Risk of death in the long QT syndrome when a sibling has died

Kaufman, E. S., McNitt, S. A., Moss, A. J., Zaręba, W., Robinson, J. L., Hall, W. J., Ackerman, M. J., Benhorin, J., Locati, E. H., Napolitano, C., et al. (2008) · Heart Rhythm · doi:10.1016/j.hrthm.2008.02.029 · Cited by 70 · see citing works

Phase 2 reentry as a mechanism of initiation of circus movement reentry in canine epicardium exposed to simulated ischemia

LUKAS, A., & Antzelevitch, C. (1996) · Cardiovascular Research · doi:10.1016/0008-6363(96)00115-0 · Cited by 58 · see citing works

Sudden infant death syndrome and long QT syndrome: an epidemiological and genetic study

Wedekind, H., Bajanowski, T., Friederich, P., et al. (2005) · International Journal of Legal Medicine · doi:10.1007/s00414-005-0019-0 · Cited by 55 · see citing works

Age‐ and Genotype‐Specific Triggers for Life‐Threatening Arrhythmia in the Genotyped Long QT Syndrome

Sakaguchi, T., Shimizu, W., Itoh, H., et al. (2008) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.2008.01138.x · Cited by 33 · see citing works

The molecular basis of catecholaminergic polymorphic ventricular tachycardia: What are the different hypotheses regarding mechanisms?

Wehrens, X. H. T. (2006) · Heart Rhythm · doi:10.1016/j.hrthm.2006.12.016 · Cited by 32 · see citing works

Cardiac arrhythmias of genetic origin are important contributors to sudden infant death syndrome

Schwartz, P. J., & Crotti, L. (2007) · Heart Rhythm · doi:10.1016/j.hrthm.2007.03.035 · Cited by 11 · see citing works

2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS

Kirchhof, P., Benussi, S., Kotecha, D., Ahlsson, A., Atar, D., Casadei, B., Castella, M., Diener, H.-C., Heidbuchel, H., Hendriks, J., et al. (2016) · European Heart Journal · doi:10.1093/eurheartj/ehw210 · Cited by 6,418 · see citing works

2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS

Kirchhof, P., Benussi, S., Kotecha, D., Ahlsson, A., Atar, D., Casadei, B., Castella, M., Diener, H.-C., Heidbuchel, H., Hendriks, J., et al. (2016) · European Journal of Cardio-Thoracic Surgery · doi:10.1093/ejcts/ezw313 · Cited by 1,403 · see citing works

Dantrolene rescues arrhythmogenic RYR2 defect in a patient‐specific stem cell model of catecholaminergic polymorphic ventricular tachycardia

Jung, C. B., Moretti, A., Mederos y Schnitzler, M., Iop, L., Storch, U., Bellin, M., Dorn, T., Ruppenthal, S., Pfeiffer, S., Goedel, A., et al. (2012) · EMBO Molecular Medicine · doi:10.1002/emmm.201100194 · Cited by 327 · see citing works

EHRA/HRS/APHRS expert consensus on ventricular arrhythmias

Pedersen, C. T., Kay, G. N., Kalman, J., Borggrefe, M., Della-Bella, P., Dickfeld, T., Dorian, P., Huikuri, H., Kim, Y.-H., Knight, B., et al. (2014) · Heart Rhythm · doi:10.1016/j.hrthm.2014.07.024 · Cited by 288 · see citing works

Genetic Arrhythmias (Channelopathies)

Mazzanti, A., Ng, K. K., & Priori, S. G. (2015) · KARGER eBooks · doi:10.1159/000375217

Neurologic complications of genetic channelopathies

Franklin, W. H., & Laubham, M. (2021) · Handbook of clinical neurology · doi:10.1016/b978-0-12-819814-8.00014-7 · Cited by 8 · see citing works

Arrhythmogenic Channelopathy Syndromes Presenting as Refractory Epilepsy

Hazle, M. A., Shellhaas, R. A., Bradley, D. J., et al. (2013) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2013.03.017 · Cited by 22 · see citing works

The Channelopathies and Sudden Death

Gomes, J. (2020) · Springer eBooks · doi:10.1007/978-3-030-45066-3_19

Ventricular Arrhythmias in Inherited Channelopathies

Issa, Z. F., Miller, J. M., & Zipes, D. P. (2019) · Elsevier eBooks · doi:10.1016/b978-0-323-52356-1.00031-1 · Cited by 3 · see citing works