Long-Term Follow-Up of Patients With Short QT Syndrome
Open accessAbstract
Objectives The aim of this study was to investigate the clinical characteristics and the long-term course of a large cohort of patients with short QT syndrome (SQTS). Background SQTS is a rare channelopathy characterized by an increased risk of sudden death. Data on the long-term outcome of SQTS patients are not available. Methods Fifty-three patients from the European Short QT Registry (75% males; median age: 26 years) were followed up for 64 ± 27 months. Results A familial or personal history of cardiac arrest was present in 89%. Sudden death was the clinical presentation in 32%. The average QTc was 314 ± 23 ms. A mutation in genes related to SQTS was found in 23% of the probands; most of them had a gain of function mutation in HERG (SQTS1). Twenty-four patients received an implantable cardioverter defibrillator, and 12 patients received long-term prophylaxis with hydroquinidine (HQ), which was effective in preventing the induction of ventricular arrhythmias. Patients with a HERG mutation had shorter QTc at baseline and a greater QTc prolongation after treatment with HQ. During follow-up, 2 already symptomatic patients received appropriate implantable cardioverter defibrillator shocks and 1 had syncope. Nonsustained polymorphic ventricular tachycardia was recorded in 3 patients. The event rate was 4.9% per year in the patients without antiarrhythmic therapy. No arrhythmic events occurred in patients receiving HQ. Conclusions SQTS carries a high risk of sudden death in all age groups. Symptomatic patients have a high risk of recurrent arrhythmic events. HQ is effective in preventing ventricular tachyarrhythmia induction and arrhythmic events during long-term follow-up.
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Quinidine therapy might provide some protection against VA in short-QT syndrome.
“Quinidine therapy might provide some protection against VA; however, there are insufficient data to make any recommendations (305,306).”
From 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope (2017) · cites this paper as [306]
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Cites
Jervell, A., & Lange-Nielsen, F. (1957) · American Heart Journal · doi:10.1016/0002-8703(57)90079-0 · Cited by 1,603 · see citing works
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 · cited 4× · see citing works
“A family had a mutation in CACNB2b (8).”
InfluentialSelf-citationSudden 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
Self-citationShort 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
Self-citationIdiopathic 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
Sex differences in the evolution of the electrocardiographic QT interval with age.
Rautaharju, P. M., Sh, Z., Wong, S., Hp, C., Berenson, G. S., Prineas, R., & Davignon, A. (1992) · PubMed · Cited by 635 · 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
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
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
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
Self-citationShort QT syndrome: Clinical findings and diagnostic-therapeutic implications
Giustetto, C., Di Monte, F., Wolpert, C., Borggrefe, M., Schimpf, R., Sbragia, P., Leone, G., Maury, P., Anttonen, O., Haissaguerre, M., et al. (2006) · European Heart Journal · doi:10.1093/eurheartj/ehl185 · Cited by 339 · see citing works
Self-citationBidoggia, H., Maciel, J. P., Capalozza, N., Mosca, S. M., Blaksley, E. J., Valverde, E. R., Bertran, G. C., Arini, P. D., Biagetti, M. O., & Quinteiro, R. A. (2000) · American Heart Journal · doi:10.1067/mhj.2000.109918 · Cited by 258 · see citing works
Further Insights into the Effect of Quinidine in Short QT Syndrome Caused by a Mutation in HERG
Wolpert, C., Schimpf, R., Giustetto, C., Antzelevitch, C. N., Cordeiro, J. M., Dumaine, R., Brugada, R., Hong, K., Bauersfeld, U., Gaïta, F., et al. (2005) · Journal of Cardiovascular Electrophysiology · doi:10.1046/j.1540-8167.2005.04470.x · Cited by 195 · see citing works
Self-citationQT interval variables from 24 hour electrocardiography and the two year risk of sudden death.
Algra, A., Tijssen, J. G. P., Roelandt, J. R. T. C., Pool, J., & Lubsen, J. C. (1993) · Heart · doi:10.1136/hrt.70.1.43 · Cited by 179 · 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
Self-citationLow Birth Weight and Increased Sympathetic Activity
Weitz, G., Fehm, H. L., & Dodt, C. (2004) · Circulation · doi:10.1161/01.cir.0000115205.70330.05 · Cited by 126 · see citing works
Gallagher, M. M., Magliano, G., Yap, Y. G., Padula, M., Morgia, V., Postorino, C., Liberato, F. D., Leo, R., Borzi, M., & Romeo, F. (2006) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2006.04.035 · Cited by 115 · see citing works
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
Self-citationA novel mutation in the KCNH2 gene associated with short QT syndrome.
Sun, Y., Quan, X.-Q., Fromme, S. J., Cox, R., Zhang, P., Zhang, L., Guo, D., Guo, J.-H., Patel, C., Kowey, P., et al. (2011) · Journal of Molecular and Cellular Cardiology · doi:10.1016/j.yjmcc.2010.11.017 · Cited by 78 · see citing works
Quinidine: a valuable medication joins the list of 'endangered species'.
Viskin, S., Antzelevitch, C., Márquez, M., & Belhassen, B. (2007) · Europace · doi:10.1093/europace/eum181 · Cited by 72 · see citing works
Anttonen, O. M., Junttila, J. M., Maury, P., Schimpf, R., Wolpert, C., Borggrefe, M., Giustetto, C., Gaïta, F., Sacher, F., Haı̈ssaguerre, M., et al. (2008) · Heart Rhythm · doi:10.1016/j.hrthm.2008.10.033 · Cited by 62 · see citing works
Self-citationSchimpf, R., Veltmann, C., Giustetto, C., Gaïta, F., Borggrefe, M., & Wolpert, C. (2007) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.2007.00925.x · Cited by 56 · see citing works
Self-citationPanicker, G. K., Karnad, D. R., Natekar, M., Kothari, S., Narula, D. D., & Lokhandwala, Y. Y. (2009) · Journal of Electrocardiology · doi:10.1016/j.jelectrocard.2009.01.003 · Cited by 53 · see citing works
Age related variation of salivary testosterone values in healthy Japanese males
Uchida, A., Bribiescas, R. G., Ellison, P. T., Kanamori, M., Ando, J., Hirose, N., & Ono, Y. (2006) · The Aging Male · doi:10.1080/13685530601060461 · Cited by 39 · see citing works
Short QT interval syndrome: a case report
Kırılmaz, A., Ulusoy, R. E., Kardeşoğlu, E., Özmen, N., & Demiralp, E. (2005) · Journal of Electrocardiology · doi:10.1016/j.jelectrocard.2005.06.093 · Cited by 15 · see citing works
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Heart Disease and Stroke Statistics—2017 Update: A Report From the American Heart Association
Benjamin, E. J., Blaha, M. J., Chiuve, S. E., Cushman, M., Das, S. R., Deo, R., de Ferranti, S. D., Floyd, J., Fornage, M., Gillespie, C., et al. (2017) · Circulation · doi:10.1161/cir.0000000000000485 · Cited by 12,591 · see citing works
Hindricks, G., Potpara, T., Dagres, N., Arbelo, E., Bax, J., Blomström-Lundqvist, C., Boriani, G., Castellá, M., Dan, G., Dilaveris, P. E., et al. (2020) · European Heart Journal · doi:10.1093/eurheartj/ehaa612 · Cited by 2,605 · see citing works
Heart Disease and Stroke Statistics—2019 Update: A Report From the American Heart Association
Benjamin, E. J., Muntner, P., Alonso, A., Bittencourt, M. S., Callaway, C. W., Carson, A. P., Chamberlain, A. M., Chang, A. R., Cheng, S., Das, S. R., et al. (2019) · Circulation · doi:10.1161/cir.0000000000000659 · Cited by 9,036 · see citing works
Heart disease and stroke statistics-2018 update: a report from the american heart association
Benjamin, E. J., Virani, S. S., Callaway, C. W., Chamberlain, A. M., Chang, A. R., Cheng, S., Chiuve, S. E., Cushman, M., Delling, F. N., Deo, R., et al. (2018) · Circulation · doi:10.1161/cir.0000000000000558 · Cited by 7,146 · see citing works
Heart Disease and Stroke Statistics—2012 Update: A Report From the American Heart Association
Writing Group Members, Roger, V. L., Go, A. S., Lloyd-Jones, D. M., Benjamin, E. J., Berry, J. D., Borden, W. B., Bravata, D. M., Dai, S., Ford, E. S., et al. (2012) · Circulation · doi:10.1161/cir.0b013e31823ac046 · Cited by 7,333 · see citing works
Heart Disease and Stroke Statistics—2016 Update: A Report From the American Heart Association
Mozaffarian, D., Benjamin, E. J., Go, A. S., Arnett, D. K., Blaha, M. J., Cushman, M., Das, S. R., de Ferranti, S., Després, J.-P., Fullerton, H. J., et al. (2016) · Circulation · doi:10.1161/cir.0000000000000350 · Cited by 6,596 · see citing works
Heart Disease and Stroke Statistics—2023 Update: A Report From the American Heart Association
Tsao, C. W., Aday, A. W., Almarzooq, Z. I., Anderson, C. A. M., Arora, P., Avery, C. L., Baker-Smith, C. M., Beaton, A. Z., Boehme, A. K., Buxton, A. E., et al. (2023) · Circulation · doi:10.1161/cir.0000000000001123 · Cited by 8,300 · see citing works
Heart Disease and Stroke Statistics—2022 Update: A Report From the American Heart Association
Tsao, C. W., Aday, A. W., Almarzooq, Z. I., Alonso, A., Beaton, A. Z., Bittencourt, M. S., Boehme, A. K., Buxton, A. E., Carson, A. P., Commodore-Mensah, Y., et al. (2022) · Circulation · doi:10.1161/cir.0000000000001052 · Cited by 5,987 · see citing works
Heart Disease and Stroke Statistics—2015 Update: A Report From the American Heart Association
Mozaffarian, D., Benjamin, E. J., Go, A. S., Arnett, D. K., Blaha, M. J., Cushman, M., de Ferranti, S., Després, J.-P., Fullerton, H. J., Howard, V. J., et al. (2015) · Circulation · doi:10.1161/cir.0000000000000152 · Cited by 5,797 · see citing works
Heart Disease and Stroke Statistics—2014 Update
Go, A. S., Mozaffarian, D., Roger, V. L., Benjamin, E. J., Berry, J. D., Blaha, M. J., Dai, S., Ford, E. S., Fox, C. S., Franco, S., et al. (2013) · Circulation · doi:10.1161/01.cir.0000441139.02102.80 · Cited by 5,207 · see citing works
Heart Disease and Stroke Statistics—2013 Update: A Report From the American Heart Association
Go, A. S., Mozaffarian, D., Roger, V. L., Benjamin, E. J., Berry, J. D., Borden, W. B., Bravata, D. M., Dai, S., Ford, E. S., Fox, C. S., et al. (2013) · Circulation · doi:10.1161/cir.0b013e31828124ad · Cited by 5,292 · see citing works
Heart Disease and Stroke Statistics—2021 Update: A Report From the American Heart Association
Virani, S. S., Alonso, A., Aparicio, H. J., Benjamin, E. J., Bittencourt, M. S., Callaway, C. W., Carson, A. P., Chamberlain, A. M., Cheng, S., Delling, F. N., et al. (2021) · Circulation · doi:10.1161/cir.0000000000000950 · Cited by 5,784 · see citing works
Priori, S. G., Blomström-Lundqvist, C., Mazzanti, A., Blom, N., Borggrefe, M., Camm, J., Elliott, P. M., Fitzsimons, D., Hatala, R., Hindricks, G., et al. (2015) · European Heart Journal · doi:10.1093/eurheartj/ehv316 · Cited by 3,893 · see citing works
Self-citationZeppenfeld, K., Tfelt‐Hansen, J., Riva, M., Winkel, B. G., Behr, E. R., Blom, N. A., Charron, P., Corrado, D., Dagres, N., de Chillou, C., et al. (2022) · European Heart Journal · doi:10.1093/eurheartj/ehac262 · Cited by 3,321 · see citing works
Gelder, I. C. V., Rienstra, M., Bunting, K. V., Casado-Arroyo, R., Caso, V., Crijns, H. J. G. M., Potter, T. J. R. D., Dwight, J., Guasti, L., Hanke, T., et al. (2024) · European Heart Journal · doi:10.1093/eurheartj/ehae176 · Cited by 3,338 · see citing works
Martin, S. S., Aday, A. W., Almarzooq, Z. I., Anderson, C. A. M., Arora, P., Avery, C. L., Baker-Smith, C. M., Barone Gibbs, B., Beaton, A. Z., Boehme, A. K., et al. (2024) · Circulation · doi:10.1161/cir.0000000000001209 · Cited by 2,800 · see citing works
Priori, S. G., Wilde, A. A., Horie, M., Cho, Y., Behr, E. R., Berul, C., Blom, N., Brugada, J., Chiang, C.-E., Huikuri, H., et al. (2013) · Heart Rhythm · doi:10.1016/j.hrthm.2013.05.014 · Cited by 1,942 · see citing works
Al‐Khatib, S. M., Stevenson, W. G., Ackerman, M. J., Bryant, W. J., Callans, D. J., Curtis, A. B., Deal, B. J., Dickfeld, T. M., Field, M. E., Fonarow, G. C., et al. (2018) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2017.10.054 · Cited by 1,883 · see citing works
Authors Task Force Members, Priori, S. G., Blomström‐Lundqvist, C., Mazzanti, A., Blom, N. A., Borggrefe, M., Camm, J. A., Elliot, P. M., Fitzsimons, D., Hatala, R., et al. (2015) · EP Europace · doi:10.1093/europace/euv319 · Cited by 1,667 · see citing works
Self-citationMartin, S. S., Aday, A. W., Allen, N. B., Almarzooq, Z., Anderson, C. A. M., Arora, P. K., Avery, C. L., Baker-Smith, C. M., Bansal, N., Beaton, A., et al. (2025) · Circulation · doi:10.1161/cir.0000000000001303 · Cited by 1,206 · see citing works
Al‐Khatib, S. M., Stevenson, W. G., Ackerman, M. J., Bryant, W. J., Callans, D. J., Curtis, A. B., Deal, B. J., Dickfeld, T. M., Field, M. E., Fonarow, G. C., et al. (2017) · Circulation · doi:10.1161/cir.0000000000000549 · Cited by 750 · see citing works
Al‐Khatib, S. M., Stevenson, W. G., Ackerman, M. J., Bryant, W. J., Callans, D. J., Curtis, A. B., Deal, B. J., Dickfeld, T. M., Field, M. E., Fonarow, G. C., et al. (2017) · Heart Rhythm · doi:10.1016/j.hrthm.2017.10.035 · Cited by 730 · see citing works
Al‐Khatib, S. M., Stevenson, W. G., Ackerman, M. J., Bryant, W. J., Callans, D. J., Curtis, A. B., Deal, B. J., Dickfeld, T. M., Field, M. E., Fonarow, G. C., et al. (2017) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2017.10.053 · Cited by 685 · see citing works
Al‐Khatib, S. M., Stevenson, W. G., Ackerman, M. J., Bryant, W. J., Callans, D. J., Curtis, A. B., Deal, B. J., Dickfeld, T. M., Field, M. E., Fonarow, G. C., et al. (2017) · Heart Rhythm · doi:10.1016/j.hrthm.2017.10.036 · Cited by 639 · see citing works
Priori, S. G., Wilde, A. A. M., Horie, M., Cho, Y., Behr, E. R., Berul, C. I., Blom, N. A., Brugada, J., Chiang, C., Huikuri, H. V., et al. (2013) · EP Europace · doi:10.1093/europace/eut272 · Cited by 626 · see citing works
2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope
Shen, W.-K., Sheldon, R. S., Benditt, D. G., Cohen, M. I., Forman, D. E., Goldberger, Z. D., Grubb, B. P., Hamdan, M. H., Krahn, A. D., Link, M. S., et al. (2017) · Heart Rhythm · doi:10.1016/j.hrthm.2017.03.004 · Cited by 484 · see citing works
Shen, W.-K., Sheldon, R. S., Benditt, D. G., Cohen, M. I., Forman, D. E., Goldberger, Z. D., Grubb, B. P., Hamdan, M. H., Krahn, A. D., Link, M. S., et al. (2017) · Circulation · doi:10.1161/cir.0000000000000499 · Cited by 453 · see citing works
Al-Khatib, S. M., Stevenson, W. G., Ackerman, M. J., Bryant, W. J., Callans, D. J., Curtis, A. B., Deal, B. J., Dickfeld, T., Field, M. E., Fonarow, G. C., et al. (2018) · Circulation · doi:10.1161/cir.0000000000000548 · Cited by 412 · see citing works
Shen, W.-K., Sheldon, R. S., Benditt, D. G., Cohen, M. I., Forman, D. E., Goldberger, Z. D., Grubb, B. P., Hamdan, M. H., Krahn, A. D., Link, M. S., et al. (2017) · Circulation · doi:10.1161/cir.0000000000000498 · Cited by 408 · see citing works
Wilde, A. A. M., Semsarian, C., Márquez, M. F., Shamloo, A. S., Ackerman, M. J., Ashley, E. A., Sternick, E. B., Barajas-Martínez, H., Behr, E. R., Bezzina, C. R., et al. (2022) · EP Europace · doi:10.1093/europace/euac030 · Cited by 399 · see citing works
2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope
Shen, W.-K., Sheldon, R. S., Benditt, D. G., Cohen, M. I., Forman, D. E., Goldberger, Z. D., Grubb, B. P., Hamdan, M. H., Krahn, A. D., Link, M. S., et al. (2017) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2017.03.003 · Cited by 327 · see citing works
EHRA/HRS/APHRS expert consensus on ventricular arrhythmias
Pedersen, C. T., Kay, G. N., Kalman, J. M., Borggrefe, M., Bella, P. E. D., Dickfeld, T. M., Dorian, P., Huikuri, H. V., Kim, Y., Knight, B. P., et al. (2014) · EP Europace · doi:10.1093/europace/euu194 · Cited by 328 · see citing works
Self-citationEHRA/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
Self-citationSchwartz, P., Ackerman, M., Antzelevitch, C., Bezzina, C., Borggrefe, M., Cuneo, B., & Wilde, A. (2020) · Nature Reviews Disease Primers · doi:10.1038/s41572-020-0188-7 · Cited by 193 · see citing works
Self-citationWilde, A. A. M., Semsarian, C., Márquez, M. F., Shamloo, A. S., Ackerman, M. J., Ashley, E. A., Sternick, E. B., Barajas-Martínez, H., Behr, E. R., Bezzina, C. R., et al. (2022) · Heart Rhythm · doi:10.1016/j.hrthm.2022.03.1225 · Cited by 281 · see citing works
Priori, S. G., Wilde, A. A. M., Horie, M., Cho, Y., Behr, E. R., Berul, C. I., Blom, N. A., Brugada, J., Chiang, C., Huikuri, H. V., et al. (2013) · Heart Rhythm · doi:10.1016/j.hrthm.2013.07.021 · Cited by 272 · see citing works
Palaniappan, L. P., Allen, N. B., Almarzooq, Z., Anderson, C. A. M., Arora, P. K., Avery, C. L., Baker-Smith, C. M., Bansal, N., Currie, M. E., Earlie, R. S., et al. (2026) · Circulation · doi:10.1161/cir.0000000000001412 · Cited by 312 · see citing works
Nordkamp, L. R. A. O., Postema, P. G., Knops, R. E., van Dijk, N., Limpens, J., Wilde, A. A. M., & de Groot, J. R. (2015) · Heart Rhythm · doi:10.1016/j.hrthm.2015.09.010 · Cited by 267 · see citing works
Varró, A., Tomek, J., Nagy, N., Virág, L., Passini, E., Rodríguez, B., & Baczkó, I. (2020) · Physiological Reviews · doi:10.1152/physrev.00024.2019 · Cited by 219 · see citing works
Priori, S., Wilde, A., Horie, M., Cho, Y., Behr, E. R., Berul, C., Blom, N., Brugada, J., Chiang, C.-E., Huikuri, H., et al. (2014) · Journal of Arrhythmia · doi:10.1016/j.joa.2013.07.002 · Cited by 651 · see citing works
Novel Insight Into the Natural History of Short QT Syndrome
Mazzanti, A., Kanthan, A., Monteforte, N., Memmi, M., Bloise, R., Novelli, V., Miceli, C., O’Rourke, S., Borio, G., Zienciuk-Krajka, A., et al. (2014) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.09.078 · Cited by 220 · see citing works
Self-citationInherited calcium channelopathies in the pathophysiology of arrhythmias
Venetucci, L., Denegri, M., Napolitano, C., & Priori, S. (2012) · Nature Reviews Cardiology · doi:10.1038/nrcardio.2012.93 · Cited by 188 · see citing works
Lampert, R., Chung, E. H., Ackerman, M. J., Arroyo, A. R., Darden, D., Deo, R., Dolan, J., Etheridge, S. P., Gray, B. R., Harmon, K. G., et al. (2024) · Heart Rhythm · doi:10.1016/j.hrthm.2024.05.018 · Cited by 167 · see citing works
Epidemiology of Arrhythmias and Sudden Cardiac Death in Asia
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Cardiac Channelopathies and Sudden Death: Recent Clinical and Genetic Advances
Fernández-Falgueras, A., Sarquella‐Brugada, G., Brugada, J., Brugada, R., & Campuzano, Ó. (2017) · Biology · doi:10.3390/biology6010007 · Cited by 146 · see citing works
Lu, H. R., Yan, G., & Gallacher, D. J. (2013) · Journal of Pharmacological and Toxicological Methods · doi:10.1016/j.vascn.2013.01.003 · Cited by 131 · see citing works
JCS/JHRS 2020 Guideline on Pharmacotherapy of Cardiac Arrhythmias
Ono, K., Iwasaki, Y., Akao, M., Ikeda, T., Ishii, K., Inden, Y., Kusano, K., Kobayashi, Y., Koretsune, Y., Sasano, T., et al. (2022) · Circulation Journal · doi:10.1253/circj.cj-20-1212 · Cited by 132 · see citing works
J-Shaped Association Between QTc Interval Duration and the Risk of Atrial Fibrillation
Nielsen, J. B., Graff, C., Pietersen, A. H., Lind, B., Struijk, J. J., OLESEN, M. S., Haunsø, S., Gerds, T. A., Svendsen, J. H., Køber, L., et al. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.03.032 · Cited by 124 · see citing works
Modeling Short QT Syndrome Using Human‐Induced Pluripotent Stem Cell–Derived Cardiomyocytes
El-Battrawy, I., Lan, H., Cyganek, L., Zhao, Z.-H., Li, X., Buljubasic, F., Lang, S., Yücel, G., Sattler, K., Zimmermann, W., et al. (2018) · Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease · doi:10.1161/jaha.117.007394 · Cited by 105 · see citing works
Self-citationHuman Induced Pluripotent Stem Cell–Derived Cardiomyocytes as Models for Cardiac Channelopathies
Garg, P., Garg, V., Shrestha, R., Sanguinetti, M. C., Kamp, T. J., & Wu, J. C. (2018) · Circulation Research · doi:10.1161/circresaha.118.311209 · Cited by 114 · see citing works
Loss-of-activity-mutation in the cardiac chloride-bicarbonate exchanger AE3 causes short QT syndrome
Thorsen, K., Dam, V. S., Kjaer-Sorensen, K., Pedersen, L. N., Skeberdis, V. A., Jurevičius, J., Treinys, R., Petersen, I. B., Nielsen, M. S., Oxvig, C., et al. (2017) · Nature Communications · doi:10.1038/s41467-017-01630-0 · Cited by 97 · see citing works
Atrial fibrillation: the role of common and rare genetic variants
Olesen, M. S., Nielsen, M. W., Haunsø, S., & Svendsen, J. H. (2014) · European Journal of Human Genetics · doi:10.1038/ejhg.2013.139 · Cited by 112 · see citing works
Long-term follow-up of a pediatric cohort with short QT syndrome.
Villafañe, J., Atallah, J., Gollob, M., Maury, P., Wolpert, C., Gebauer, R., Watanabe, H., Horie, M., Anttonen, O., Kannankeril, P., et al. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2012.12.025 · Cited by 95 · see citing works
Self-citationPotassium-channel mutations and cardiac arrhythmias—diagnosis and therapy
Giudicessi, J. R., & Ackerman, M. J. (2012) · Nature Reviews Cardiology · doi:10.1038/nrcardio.2012.3 · Cited by 101 · see citing works
Lenarczyk, R., Zeppenfeld, K., Tfelt-Hansen, J., Heinzel, F., Deneke, T., Ene, E., Meyer, C., Wilde, A., Arbelo, E., Jędrzejczyk-Patej, E., et al. (2024) · Europace · doi:10.1093/europace/euae049 · Cited by 102 · see citing works
Self-citationMerino, J. L., Tamargo, J. L., Blomström‐Lundqvist, C., Boriani, G., Crijns, H. J. G. M., Dobrev, D., Goette, A., Hohnloser, S. H., Naccarelli, G. V., Reiffel, J. A., et al. (2025) · EP Europace · doi:10.1093/europace/euaf076 · Cited by 105 · see citing works
Heidbüchel, H., Arbelo, E., D’Ascenzi, F., Börjesson, M., Bovéda, S., Castelletti, S., Miljoen, H., Mont, L., Niebauer, J., Papadakis, M., et al. (2020) · EP Europace · doi:10.1093/europace/euaa106 · Cited by 93 · see citing works
Priori, S. G., Wilde, A. A. M., Horie, M., Cho, Y., Behr, E. R., Berul, C. I., Blom, N. A., Brugada, J., Chiang, C., Huikuri, H. V., et al. (2013) · Journal of Arrhythmia · doi:10.1016/j.joa.2013.08.001 · Cited by 90 · see citing works
Shinnawi, R., Shaheen, N., Huber, I., Shiti, A., Arbel, G., Gepstein, A., Ballan, N., Setter, N., Tijsen, A. J., Borggrefe, M. M., et al. (2019) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2019.02.055 · Cited by 89 · see citing works
Self-citationHydroquinidine Prevents Life-Threatening Arrhythmic Events in Patients With Short QT Syndrome
Mazzanti, A., Maragna, R., Vacanti, G., Κωστοπούλου, Ά., Marino, M., Monteforte, N., Bloise, R., Underwood, K., Tibollo, V., Pagan, E., et al. (2017) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2017.10.025 · Cited by 89 · see citing works
Self-citationAtrial Fibrillation Predictors: Importance of the Electrocardiogram
German, D. M., Kabir, M., Dewland, T. A., Henrikson, C. A., & Tereshchenko, L. G. (2015) · Annals of Noninvasive Electrocardiology · doi:10.1111/anec.12321 · Cited by 85 · see citing works
Dhutia, H., Malhotra, A., Parpia, S., Gabus, V., Finocchiaro, G., Mellor, G. J., Merghani, A., Millar, L. M., Narain, R., Sheikh, N., et al. (2015) · British Journal of Sports Medicine · doi:10.1136/bjsports-2015-094827 · Cited by 82 · see citing works
Recent Advances in Short QT Syndrome
Campuzano, Ó., Sarquella-Brugada, G., César, S., Arbelo, E., Brugada, J., & Brugada, R. (2018) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2018.00149 · Cited by 69 · see citing works
Ventricular arrhythmias. Ventricular tachycardias and sudden cardiac death. 2020 Clinical guidelines
Lebedev, D. S., Mikhaylov, E. N., Neminuschiy, N. M., Golukhova, E. Z., Babokin, V. E., Bereznitskaya, V. V., Vasichkina, E. S., Garkina, S. V., Sp, G., Davtyan, K. V., et al. (2021) · Russian Journal of Cardiology · doi:10.15829/1560-4071-2021-4600 · Cited by 86 · see citing works
Mutations in voltage-gated L-type calcium channel: implications in cardiac arrhythmia
Zhang, Q., Chen, J.-J., Qin, Y., Wang, J.-J., & Zhou, L. (2018) · Channels · doi:10.1080/19336950.2018.1499368 · Cited by 74 · see citing works
Short QT Interval Prevalence and Clinical Outcomes in a Pediatric Population
Guerrier, K., Kwiatkowski, D., Czosek, R., Spar, D., Anderson, J. B., & Knilans, T. (2015) · Circulation: Arrhythmia and Electrophysiology · doi:10.1161/circep.115.003256 · Cited by 57 · see citing works
Harrell, D. T., Ashihara, T., Ishikawa, T., Tominaga, I., Mazzanti, A., Takahashi, K., Oginosawa, Y., Abe, H., Maemura, K., Sumitomo, N., et al. (2015) · International Journal of Cardiology · doi:10.1016/j.ijcard.2015.04.090 · Cited by 64 · see citing works
Self-citationEpidemiology of inherited arrhythmias
Offerhaus, J. A., Bezzina, C. R., & Wilde, A. A. M. (2019) · Nature Reviews Cardiology · doi:10.1038/s41569-019-0266-2 · Cited by 66 · see citing works
Cardiac Delayed Rectifier Potassium Channels in Health and Disease
Chen, L., Sampson, K. J., & Kass, R. S. (2016) · Cardiac Electrophysiology Clinics · doi:10.1016/j.ccep.2016.01.004 · Cited by 64 · see citing works
Diagnosis and management of short QT syndrome.
Bjerregaard, P. (2018) · Heart Rhythm · doi:10.1016/j.hrthm.2018.02.034 · Cited by 52 · see citing works
Drug-induced QT-interval shortening following antiepileptic treatment with oral rufinamide
Schimpf, R., Veltmann, C., Papavassiliu, T., Rudic, B., Göksu, T., Kuschyk, J., Wolpert, C., Antzelevitch, C., Ebner, A., Borggrefe, M., et al. (2012) · Heart Rhythm · doi:10.1016/j.hrthm.2012.01.006 · Cited by 60 · see citing works
Self-citationWilde, A. A. M., Semsarian, C., Márquez, M. F., Shamloo, A. S., Ackerman, M. J., Ashley, E. A., Sternick, E. B., Barajas-Martínez, H., Behr, E. R., Bezzina, C. R., et al. (2022) · Journal of Arrhythmia · doi:10.1002/joa3.12717 · Cited by 64 · see citing works
Moreno, C., Oliveras, A., de la Cruz, A., Bartolucci, C., Muñoz, C., Salar, E., Gimeno, J., Severi, S., Comes, N., Felipe, A., et al. (2015) · Cardiovascular Research · doi:10.1093/cvr/cvv196 · Cited by 58 · see citing works
Short QT Syndrome - Review of Diagnosis and Treatment.
Rudic, B., Schimpf, R., & Borggrefe, M. (2014) · Arrhythmia & Electrophysiology Review · doi:10.15420/aer.2014.3.2.76 · Cited by 55 · see citing works
Self-citationThe Phenotypic Spectrum of a Mutation Hotspot Responsible for the Short QT Syndrome.
Hu, D., Li, Y., Zhang, J.-C., Pfeiffer, R., Gollob, M., Healey, J., Harrell, D. T., Makita, N., Abe, H., Sun, Y., et al. (2017) · JACC Clinical Electrophysiology · doi:10.1016/j.jacep.2016.11.013 · Cited by 53 · see citing works
Self-citationLong‐Term Follow‐Up of Patients With Short QT Syndrome: Clinical Profile and Outcome
El-Battrawy, I., Besler, J., Liebe, V., Schimpf, R., Tülümen, E., Rudic, B., Lang, S., Wolpert, C., Zhou, X., Akin, I., et al. (2018) · Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease · doi:10.1161/jaha.118.010073 · Cited by 39 · see citing works
Self-citationGenetics of ion-channel disorders
Cerrone, M., Napolitano, C., & Priori, S. G. (2012) · Current Opinion in Cardiology · doi:10.1097/hco.0b013e328352429d · Cited by 49 · see citing works
Du, C., Zhang, Y., Harchi, A. E., Dempsey, C. E., & Hancox, J. C. (2014) · Journal of Molecular and Cellular Cardiology · doi:10.1016/j.yjmcc.2014.05.013 · Cited by 49 · see citing works
Electrical Storm in Short‐QT Syndrome Successfully Treated with Isoproterenol
Bun, S., Maury, P., Giustetto, C., & Deharo, J. (2012) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.2012.02295.x · Cited by 48 · see citing works
Self-citationPrecision Medicine and cardiac channelopathies: when dreams meet reality
Gnecchi, M., Sala, L., & Schwartz, P. J. (2021) · European Heart Journal · doi:10.1093/eurheartj/ehab007 · Cited by 44 · see citing works
Emerging therapeutic targets in the short QT syndrome
Hancox, J., Whittaker, D. G., Du, C., Stuart, A. G., & Zhang, H.-G. (2018) · Expert opinion on therapeutic targets · doi:10.1080/14728222.2018.1470621 · Cited by 48 · see citing works
Usefulness of exercise test in the diagnosis of short QT syndrome.
Giustetto, C., Scrocco, C., Schimpf, R., Maury, P., Mazzanti, A., Levetto, M., Anttonen, O., Dalmasso, P., Cerrato, N., Gribaudo, E., et al. (2015) · Europace · doi:10.1093/europace/euu351 · Cited by 39 · see citing works
Self-citationMazzanti, A., O’Rourke, S., Ng, K., Miceli, C., Borio, G., Curcio, A., Esposito, F., Napolitano, C., & Priori, S. (2014) · Expert Review of Cardiovascular Therapy · doi:10.1586/14779072.2014.894884 · Cited by 46 · see citing works
Self-citationOptimal antiarrhythmic drug therapy for electrical storm
Sorajja, D., Munger, T. M., & Shen, W.-K. (2015) · Journal of Biomedical Research · doi:10.7555/jbr.29.20140147 · Cited by 44 · see citing works
EHRA/HRS/APHRS expert consensus on ventricular arrhythmias
Pedersen, C. T., Kay, G. N., Kalman, J. M., Borggrefe, M., Bella, P. E. D., Dickfeld, T. M., Dorian, P., Huikuri, H. V., Kim, Y. D., Knight, B. P., et al. (2014) · Journal of Arrhythmia · doi:10.1016/j.joa.2014.08.001 · Cited by 44 · see citing works
Self-citationJCS / JHRS 2020 Guideline on Pharmacotherapy of Cardiac Arrhythmias
Ono, K., Iwasaki, Y., Akao, M., Ikeda, T., Ishii, K., Inden, Y., Kusano, K., Kobayashi, Y., Koretsune, Y., Sasano, T., et al. (2022) · Journal of Arrhythmia · doi:10.1002/joa3.12714 · Cited by 41 · see citing works
Menafoglio, A., Valentino, M. D., Segatto, J.-M., Siragusa, P., Pezzoli, R., Maggi, M., Romano, G. A., Moschovitis, G., Wilhelm, M., & Gallino, A. (2014) · British Journal of Sports Medicine · doi:10.1136/bjsports-2013-092929 · Cited by 42 · see citing works
Electrocardiogram (ECG) for the Prediction of Incident Atrial Fibrillation: An Overview
Aizawa, Y. (2017) · Journal of Atrial Fibrillation · doi:10.4022/jafib.1724 · Cited by 42 · see citing works
Short QT in a Cohort of 1.7 Million Persons: Prevalence, Correlates, and Prognosis
Iribarren, C., Round, A. D., Peng, J. A., Lü, M., Klatsky, A. L., Zaroff, J. G., Holve, T. J., Prasad, A., & Stang, P. (2014) · Annals of Noninvasive Electrocardiology · doi:10.1111/anec.12157 · Cited by 41 · see citing works
New echocardiographic insights in short QT syndrome: More than a channelopathy?
Frea, S., Giustetto, C., Capriolo, M., Scrocco, C., Fornengo, C., Benedetto, S., Bianchi, F., Pidello, S., Morello, M., & Gaïta, F. (2015) · Heart Rhythm · doi:10.1016/j.hrthm.2015.05.024 · Cited by 41 · see citing works
Self-citationImplantable defibrillators in primary prevention of genetic arrhythmias. A shocking choice?
Corrado, D., Link, M. S., & Schwartz, P. J. (2022) · European Heart Journal · doi:10.1093/eurheartj/ehac298 · Cited by 41 · see citing works
Genética y arritmias: aplicaciones diagnósticas y pronósticas
Monteforte, N., Napolitano, C., & Priori, S. G. (2012) · Revista Española de Cardiología · doi:10.1016/j.recesp.2011.10.008 · Cited by 39 · see citing works
Karathanos, T. V., Boyle, P. M., & Trayanova, N. A. (2014) · EP Europace · doi:10.1093/europace/euu250 · Cited by 40 · see citing works
Atrial fibrillation in inherited cardiac channelopathies: From mechanisms to management.
Enriquez, A., Antzelevitch, C., Bismah, V., & Baranchuk, A. (2016) · Heart Rhythm · doi:10.1016/j.hrthm.2016.06.008 · Cited by 38 · see citing works
The new kids on the block of arrhythmogenic disorders: Short QT syndrome and early repolarization
Mazzanti, A., Underwood, K., Nevelev, D., Kofman, S., & Priori, S. (2017) · Cardiovascular Electrophysiology · doi:10.1111/jce.13265 · Cited by 32 · see citing works
Self-citationImpact of Antiarrhythmic Drugs on the Outcome of Short QT Syndrome
El‐Battrawy, I., Besler, J., Li, X., Lan, H., Zhao, Z., Liebe, V., Schimpf, R., Lang, S., Wolpert, C., Zhou, X., et al. (2019) · Frontiers in Pharmacology · doi:10.3389/fphar.2019.00771 · Cited by 38 · see citing works
Self-citationManagement of Ventricular Arrhythmias in Suspected Channelopathies
Obeyesekere, M., Antzelevitch, C., & Krahn, A. (2015) · Circulation: Arrhythmia and Electrophysiology · doi:10.1161/circep.114.002321 · Cited by 34 · see citing works
Recommendations for the use of electrophysiological study: Update 2018
Mureşan, L., Cismaru, G. L., Martins, R. P., Bataglia, A., Roşu, R., Puiu, M., Guşetu, G., Mada, R. O., Muresan, C., Ispas, D. R., et al. (2018) · Hellenic Journal of Cardiology · doi:10.1016/j.hjc.2018.09.002 · Cited by 37 · see citing works
Implantable cardioverter‐defibrillator in Brugada syndrome: Long‐term follow‐up
El‐Battrawy, I., Roterberg, G., Liebe, V., Ansari, U., Lang, S., Zhou, X., Borggrefe, M. M., & Akın, İ. (2019) · Clinical Cardiology · doi:10.1002/clc.23247 · Cited by 36 · see citing works
Self-citation
- Authors
- Giustetto, C., Schimpf, R., Mazzanti, A., Scrocco, C., Maury, P., Anttonen, O., Probst, V., Blanc, J.-J., Sbragia, P., Dalmasso, P., Borggrefe, M., & Gaita, F.
- Affiliations
- Azienda Ospedaliero Universitaria San Giovanni Battista (IT)
- University Medical Centre Mannheim (DE)
- Hôpital Rangueil (FR)
- Päijät-Hämeen Keskussairaala (FI)
- Institut du Thorax (FR)
- Université de Bretagne Occidentale (FR)
- Hôpital Nord (FR)
- University of Turin (IT)
- Corresponding author
- Giustetto, Carla
- ORCID
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 2011-08
- Volume
- 58
- Issue
- 6
- Pages
- 587-595
- ISSN
- 0735-1097 (ISSN-L)
- OpenAlex
- W2006047900
- Cited by
- 325
- References
- 25
- Citations by year
- 2026: 10 · 2025: 11 · 2024: 20 · 2023: 14 · 2022: 14 · 2021: 17 · 2020: 26 · 2019: 21 · 2018: 26 · 2017: 32 · 2016: 16 · 2015: 33 · 2014: 21 · 2013: 31 · 2012: 28
- FWCI
- 18.70
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion, other-oa)
- Funders
- Foundation Cassa di Risparmio of Asti
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Keywords
- short QT syndrome, sudden cardiac death, sudden cardiac arrest, gain-of-function mutations, QTc prolongation, hydroquinidine, implantable cardioverter-defibrillator, ventricular tachyarrhythmia, long-term follow-up, channelopathies, antiarrhythmic prophylaxis
- MeSH terms
- ERG1 Potassium Channel, Adolescent, Adult, Anti-Arrhythmia Agents, Arrhythmias, Cardiac, Death, Sudden, Electrocardiography, Female, Follow-Up Studies, Humans, Male, Middle Aged, Mutation, Quinidine, Syncope, Cohort Studies, Treatment Outcome, Defibrillators, Implantable, Ether-A-Go-Go Potassium Channels
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