The DOI matches its Crossref record; 25 of the 25 references it cites were found in a registry or an index.
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; 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 1,601
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×
“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
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
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
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
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
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
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
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
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Scrocco, C., Gaïta, F., & Giustetto, C. (2021) · Springer eBooks · doi:10.1007/978-3-030-80447-3_10
Self-citationHancox, J. C., McPate, M. J., Harchi, A. E., Butler, A. S., Zhang, Y., Whittaker, D. J., Dempsey, C. E., Witchel, H. J., Adeniran, I., Stuart, G., et al. (2023) · Springer eBooks · doi:10.1007/978-3-031-33588-4_26 · Cited by 1
Ifedili, I., Maturana, M. A., Kayali, S. M., Levine, Y. C., Kabra, R., & Jha, S. K. (2024) · Journal of Cardiovascular Electrophysiology · doi:10.1111/jce.16164
Rudic, B., & Borggrefe, M. (2024) · Springer Reference Medizin · doi:10.1007/978-3-642-54676-1_178-2
Self-citationFanous, Y., & Roberts, J. D. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-15734-9.00006-6
Kean, A. C., & Kannankeril, P. J. (2024) · Springer eBooks · doi:10.1007/978-3-031-07563-6_47
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Arslan, G. Y., Hoşoğlu, Y., Hoşoğlu, A., Yiğit, Y., Akgün, D. E., Akgün, Y. S., Yıldırım, T., Yıldırım, S. E., Yazar, A., Danışman, N., et al. (2024) · Akademisyen Kitabevi eBooks · doi:10.37609/akya.3125
Arbelo, E., & Sánchez-Giménez, R. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-22344-0.00012-7
Class I Antiarrhythmic Drugs: Na+ Channel Blockers
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Short QT Syndrome and Drug Treatment: A systematic Literature Review and PRISMA Analysis
You, T., Luo, C.-J., Zhang, K., & Zhang, H.-G. (2025) · International Journal of Drug Discovery and Pharmacology · doi:10.53941/ijddp.2025.100009 · Cited by 1
Tan, L., Liu, J., Guo, F., Lin, J., Xiong, R., Fu, H., You, H.-M., He, L., Xia, S., Du, X., et al. (2025) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2025.1555512
Belhassen, B., & Milman, A. (2025) · Indian Pacing and Electrophysiology Journal · doi:10.1016/j.ipej.2025.07.018
RetractedBoyd, C. A., Novy, T., Beecroft, T., Bell, C. A., Bland, E. M., & Miyake, C. Y. (2025) · HeartRhythm Case Reports · doi:10.1016/j.hrcr.2025.10.006
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Firouzi, A., Khalilipur, E., & Hosseini, Z. (2025) · Springer eBooks · doi:10.1007/978-3-031-97321-5_6
A case of a familial ST segment depression syndrome
Фурман, Н. В., Malenova, A. Y., Petrova, N. S., & Patsura, O. Y. (2026) · Russian Journal of Preventive Medicine · doi:10.17116/profmed20262904191
Familial Short QT Syndrome: Phenotypic Variability and Challenges in Risk Stratification
Bouzón, P., Alén, A., Salgado, M., González-Urbistondo, F., Vega-Prado, L. M., Coto, E., Rodríguez-Reguero, J. J., Gomez, J., Barrio, B. F., y, P. A., et al. (2026) · Journal of Clinical Medicine · doi:10.3390/jcm15093461
Short QT syndrome: From electrocardiogram to therapy
Gaïta, F., & Borggrefe, M. M. (2026) · Heart Rhythm · doi:10.1016/j.hrthm.2026.02.055
Self-citationBazoukis, G., & Saplaouras, A. (2026) · JACC Case Reports · doi:10.1016/j.jaccas.2026.108482
Sarquella‐Brugada, G., Mazzanti, A., Baban, A., Campuzano, Ó., Conte, G., Crotti, L., Ackerman, M. J., Behr, E. R., Blom, N. A., Chowdhury, D., et al. (2026) · EP Europace · doi:10.1093/europace/euag184
Self-citationСнежицкий, В. А. (2026) · Кардиология в Беларуси · doi:10.34883/pi.2026.18.5.009
Metadata
- 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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