The DOI matches its Crossref record; 21 of the 21 references it cites were found in a registry or an index.
Short QT Syndrome: A Familial Cause of Sudden Death
Abstract
Background— A prolonged QT interval is associated with a risk for life-threatening events. However, little is known about prognostic implications of the reverse—a short QT interval. Several members of 2 different families were referred for syncope, palpitations, and resuscitated cardiac arrest in the presence of a positive family history for sudden cardiac death. Autopsy did not reveal any structural heart disease. All patients had a constantly and uniformly short QT interval at ECG. Methods and Results— Six patients from both families were submitted to extensive noninvasive and invasive work-up, including serial resting ECGs, echocardiogram, cardiac MRI, exercise testing, Holter ECG, and signal-averaged ECG. Four of 6 patients underwent electrophysiological evaluation including programmed ventricular stimulation. In all subjects, a structural heart disease was excluded. At baseline ECG, all patients exhibited a QT interval ≤280 ms (QTc ≤300 ms). During electrophysiological study, short atrial and ventricular refractory periods were documented in all and increased ventricular vulnerability to fibrillation in 3 of 4 patients. Conclusions— The short QT syndrome is characterized by familial sudden death, short refractory periods, and inducible ventricular fibrillation. It is important to recognize this ECG pattern because it is related to a high risk of sudden death in young, otherwise healthy subjects.
Overview
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“Invasive EPS has shown increased vulnerability to VF induction in most patients, yet the clinical significance of this finding remains unknown (305).”
From 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope (2017) · cites this paper as [305]
In the citing paper
“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 [305]
In the citing paper
“Short QT syndrome KCNH2 KCNQ1 KCNJ2 CACNA1C CACNB2b IKr IKs IK1 ICa ICa Up to 70% 853, 856-858”
From 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS (2016) · cites this paper as [857]
In the citing paper
“AF prevalence ranges from 5% to 20% in patients with long QT syndrome or Brugada syndrome, and is up to 70% in short QT syndrome (Table 20).853,[856][857][858]”
From 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS (2016) · cites this paper as [857]
Citations
Cites
Brugada, P., & Brugada, J. (1992) · Journal of the American College of Cardiology · doi:10.1016/0735-1097(92)90253-j · Cited by 3,344
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
Jervell, A., & Lange-Nielsen, F. (1957) · American Heart Journal · doi:10.1016/0002-8703(57)90079-0 · Cited by 1,601
KCNQ1 Gain-of-Function Mutation in Familial Atrial Fibrillation
Chen, Y.-H., Xu, S.-J., Bendahhou, S., Wang, X.-L., Wang, Y., Xu, W.-Y., Jin, H., Sun, H., Su, X.-Y., Zhuang, Q.-N., et al. (2003) · Science · doi:10.1126/science.1077771 · Cited by 989
Proposed Diagnostic Criteria for the Brugada Syndrome
Wilde, A. A. M., Antzelevitch, C. N., Borggrefe, M. M., Brugada, J., Brugada, R., Brugada, P., Corrado, D., Hauer, R. N. W., Kass, R. S., Nademanee, K., et al. (2002) · Circulation · doi:10.1161/01.cir.0000034169.45752.4a · Cited by 844
Self-citationA NEW FAMILIAL CARDIAC SYNDROME IN CHILDREN.
Ward, O. C. (1964) · PubMed · Cited by 826
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
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
Brugada, J., Brugada, R., Antzelevitch, C. N., Towbin, J. A., Nademanee, K., & Brugada, P. (2002) · Circulation · doi:10.1161/hc0102.101354 · Cited by 624
Identification of a Genetic Locus for Familial Atrial Fibrillation
Brugada, R., Tapscott, T., Czernuszewicz, G. Z., Marian, A. J., Iglesias, A., Mont, L., Brugada, J., Girona, J. M., Domingo, A., Bachinski, L. L., et al. (1997) · New England Journal of Medicine · doi:10.1056/nejm199703273361302 · Cited by 554
Research works printed but not linked: 9
Cited by
Sorted by citations
Hindricks, G., Potpara, T., Dagres, N., Arbelo, E., Bax, J. J., Blomström‐Lundqvist, C., Boriani, G., Castella, M., Dan, G.-A., Dilaveris, P., et al. (2020) · European Heart Journal · doi:10.1093/eurheartj/ehaa612 · Cited by 10,550
2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS
Kirchhof, P. F., Benussi, S., Kotecha, D., Ahlsson, A., Atar, D., Casadei, B., Castellà, M., Diener, H., Heidbüchel, H., Hendriks, J., et al. (2016) · EP Europace · doi:10.1093/europace/euw295 · Cited by 6,438
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
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
Self-citationGelder, 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 3,594
Metadata
- Authors
- Gaita, F., Giustetto, C., Bianchi, F., Wolpert, C., Schimpf, R., Riccardi, R., Grossi, S., Richiardi, E., & Borggrefe, M.
- Affiliations
- From the Division of Cardiology (F.G., C.G., F.B., R.R., S.G., E.R.), Ospedale Mauriziano Umberto I, Torino, Italy; and the 1st Department of Medicine (C.W., R.S., M.B.), University Hospital Mannheim, University of Heidelberg, Mannheim, Germany.
- Heidelberg University (DE)
- Venue
- Circulation
- Published
- 2003-08-26
- Volume
- 108
- Issue
- 8
- Pages
- 965-970
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W1967867484
- Cited by
- 708
- References
- 21
- Citations by year
- 2026: 8 · 2025: 12 · 2024: 17 · 2023: 10 · 2022: 14 · 2021: 20 · 2020: 20 · 2019: 15 · 2018: 18 · 2017: 33 · 2016: 25 · 2015: 27 · 2014: 27 · 2013: 49 · 2012: 44
- FWCI
- 30.09
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Keywords
- short QT syndrome, short QT interval, sudden cardiac death, familial sudden death, ventricular refractory period, ventricular fibrillation, syncope, palpitations, cardiac arrest, programmed ventricular stimulation, ventricular vulnerability
- MeSH terms
- Adolescent, Adult, Aged, Arrhythmias, Cardiac, Atrial Fibrillation, Child, Electrocardiography, Exercise Test, Cardiac Complexes, Premature, Female, Flecainide, Heart Arrest, Humans, Male, Pedigree, Syncope, Syndrome, Sodium Channels, Electrocardiography, Ambulatory, Death, Sudden, Cardiac, Electrophysiologic Techniques, Cardiac
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
