Electrocardiographic Biomarkers of Ventricular Repolarisation in a Single Family of Short QT Syndrome and the Role of the Bazett Correction Formula
Abstract
The definition of the short QT syndrome (SQTS) is based on QT duration, but thorough QT- and T-wave evaluation has not been performed to date. To evaluate the influence of QT rate-correction formulas in SQTS diagnosis, 12-lead electrocardiograms (ECGs) were recorded in 27 subjects from a single family with SQTS. Based on QT duration corrected by Bazett formula (QTc), 4 men were considered to have SQTS (QTc <or=340 ms), and 16 were classified to have "suspected" SQTS (QT <or=320 ms and/or QTc <or=380 ms). Holter ECGs were recorded in the 4 patients with SQTS, 8 with suspected SQTS, and 62 healthy volunteers. We evaluated QT duration at each RR interval and calculated individual alpha coefficient of the QT/RR relation. The QT interval duration at the mean Holter heart rate was corrected using an individual approach and 5 "universal" correction formulas (Bazett, Fridericia, Sagie-Framingham, Hodges, Rautaharju). Patients with SQTS and controls had lower alpha coefficients of the QT/RR relation when compared with Bazett and Fridericia formulas. The QT rate-dependence was lower in patients with SQTS (alpha 0.146 +/- 0.070) when compared with control subjects (alpha 0.203 +/- 0.039, p <0.05). The universal correction-formulas consistently provided higher values of corrected QT interval duration at the mean Holter heart rate than a subject-specific correction formula (p <0.05), which induced false negative diagnoses. In conclusion, the Bazett correction formula is not appropriate for making a diagnosis of SQTS. The subject-specific correction formula may provide a better cut-off value definition for the diagnosis of SQTS.
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A subject-specific correction formula may provide a better cutoff value definition for the diagnosis of short QT syndrome.
“the authors suggested that this formula may provide a better cutoff value definition for the diagnosis of SQTS.”
From The Year of 2008 in Electrocardiology (2010) · cites this paper as [36]
In a single family with short QT syndrome, universal correction formulas consistently provided higher values of corrected QT interval duration at the mean Holter heart rate than a subject-specific correction formula.
“Studying a single family with SQTS, Maison-Blanche and coworkers 36 demonstrated that the universal correction-formulas consistently provided higher values of corrected QT interval duration at the mean Holter heart rate than a subjectspecific correction formula; the authors suggested that this formula may provide a better cutoff value definition for the diagnosis of SQTS.”
From The Year of 2008 in Electrocardiology (2010) · cites this paper as [36]
Cites
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
An improved method for adjusting the QT interval for heart rate (the Framingham Heart Study)
Sagie, A., Larson, M. G., Goldberg, R. J., Bengtson, J. R., & Levy, D. A. (1992) · The American Journal of Cardiology · doi:10.1016/0002-9149(92)90562-d · Cited by 715 · 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
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
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
Malík, M., Färbom, P., Batchvarov, V., Hnatkova, K., & Camm, A. J. (2002) · Heart · doi:10.1136/heart.87.3.220 · Cited by 437 · see citing works
Rate-corrected QT interval: Techniques and limitations
Funck‐Brentano, C., & Jaillon, P. (1993) · The American Journal of Cardiology · doi:10.1016/0002-9149(93)90035-b · Cited by 401 · see citing works
Electrocardiographic reference ranges derived from 79,743 ambulatory subjects
Mason, J. W., Ramseth, D. J., CHANTER, D. O., Moon, T. E., Goodman, D. B., & Mendzelevski, B. (2007) · Journal of Electrocardiology · doi:10.1016/j.jelectrocard.2006.09.003 · Cited by 387 · see citing works
Effects of I Kr and I Ks Heterogeneity on Action Potential Duration and Its Rate Dependence
Viswanathan, P. C., Shaw, R. M., & Rudy, Y. (1999) · Circulation · doi:10.1161/01.cir.99.18.2466 · Cited by 361 · see citing works
Short 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-citationBatchvarov, V. N., Ghuran, A., Smetana, P., Hnatkova, K., Harries, M., Dilaveris, P., Camm, A. J., & Malík, M. (2002) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.00860.2001 · Cited by 219 · see citing works
Extramiana, F., & Antzelevitch, C. N. (2004) · Circulation · doi:10.1161/01.cir.0000143078.48699.0c · Cited by 207 · see citing works
Self-citationFurther 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
Schimpf, R., Wolpert, C., Gaïta, F., Giustetto, C., & Borggrefe, M. (2005) · Cardiovascular Research · doi:10.1016/j.cardiores.2005.03.026 · Cited by 184 · see citing works
Viskin, S., Zeltser, D., Ish‐Shalom, M., Katz, A., Glikson, M., Justo, D., Tekes-Manova, D., & Belhassen, B. (2004) · Heart Rhythm · doi:10.1016/j.hrthm.2004.07.010 · Cited by 127 · see citing works
Rautaharju, P. M., Warren, J. W., & Calhoun, H. P. (1990) · Journal of Electrocardiology · doi:10.1016/0022-0736(90)90085-g · Cited by 116 · see citing works
Malik, M. (2002) · Pacing and Clinical Electrophysiology · doi:10.1046/j.1460-9592.2002.00209.x · Cited by 93 · see citing works
QT interval analysis on ambulatory electrocardiogram recordings: a selective beat averaging approach
Badilini, F., Maison‐Blanche, P., Childers, R. W., & Coumel, P. (1999) · Medical & Biological Engineering & Computing · doi:10.1007/bf02513269 · Cited by 92 · see citing works
Self-citationNormal limits of the electrocardiogram in Chinese subjects
Wu, J., Kors, J. A., Rijnbeek, P. R., van Herpen, G., Zaiying, L., & Xu, C. (2002) · International Journal of Cardiology · doi:10.1016/s0167-5273(02)00248-6 · Cited by 85 · see citing works
Borggrefe, M. M., Wolpert, C., Antzelevitch, C. N., Veltmann, C., Giustetto, C., Gaïta, F., & Schimpf, R. (2005) · Journal of Electrocardiology · doi:10.1016/j.jelectrocard.2005.06.009 · Cited by 76 · see citing works
Clinical assessment of drug-induced QT prolongation in association with heart rate changes
Extramiana, F., Maison‐Blanche, P., CABANIS, M., Ortemann‐Renon, C., Beaufils, P., & Leenhardt, A. (2005) · Clinical Pharmacology & Therapeutics · doi:10.1016/j.clpt.2004.10.016 · Cited by 68 · see citing works
Self-citationECG Phenomenon of Idiopathic and Paradoxical Short QT Intervals
Gussak, I. B., Brugada, P., Brugada, J., Antzelevitch, C. N., Osbakken, M. D., & Bjerregaard, P. (2002) · Cardiac Electrophysiology Review · doi:10.1023/a:1017931020747 · Cited by 62 · see citing works
Clinical characteristics and treatment of short QT syndrome
Wolpert, C., Schimpf, R., Veltmann, C., Giustetto, C., Gaïta, F., & Borggrefe, M. M. (2005) · Expert Review of Cardiovascular Therapy · doi:10.1586/14779072.3.4.611 · Cited by 42 · see citing works
Cardiac safety of noncardiac drugs : practical guidelines for clinical research and drug development
Morganroth, J., & Gussak, I. B. (2005) · DIAL (Catholic University of Leuven) · doi:10.1007/978-1-59259-884-7 · Cited by 29 · see citing works
Extramiana, F., Denjoy, I., Badilini, F., Chabani, I., Neyroud, N., Berthet, M., Fressard, V., Guicheney, P., Beaufils, P., Leenhardt, A., et al. (2005) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2004.09.045 · Cited by 17 · see citing works
Self-citationContrasting Time‐ and Rate‐Based Approaches for the Assessment of Drug‐Induced QT Changes
Extramiana, F., Badilini, F., Sarapa, N., Leenhardt, A., & Maison‐Blanche, P. (2007) · The Journal of Clinical Pharmacology · doi:10.1177/0091270007302953 · Cited by 13 · see citing works
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The short QT syndrome: proposed diagnostic criteria.
Gollob, M., Redpath, C., & Roberts, J. D. (2011) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2010.09.048 · Cited by 330 · see citing works
High prevalence of early repolarization in short QT syndrome.
Watanabe, H., Makiyama, T., Koyama, T., Kannankeril, P., Seto, S., Okamura, K., Oda, H., Itoh, H., Okada, M., Tanabe, N., et al. (2010) · Heart Rhythm · doi:10.1016/j.hrthm.2010.01.012 · Cited by 160 · see citing works
The QT interval: too long, too short or just right.
Viskin, S. (2009) · Heart Rhythm · doi:10.1016/j.hrthm.2009.02.044 · Cited by 177 · see citing works
Shah, R. (2010) · British Journal of Pharmacology · doi:10.1111/j.1476-5381.2009.00191.x · Cited by 103 · see citing works
Long QT syndrome and short QT syndrome
Zareba, W., & Cygankiewicz, I. (2008) · Progress in Cardiovascular Diseases · doi:10.1016/j.pcad.2008.10.006 · Cited by 96 · 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
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Modi, S. S., & Krahn, A. D. (2011) · Circulation · doi:10.1161/circulationaha.110.981381 · Cited by 61 · see citing works
Holbrook, M., Malik, M., Shah, R., & Valentin, J. (2009) · Journal of pharmacological and toxicological methods · doi:10.1016/j.vascn.2008.09.001 · Cited by 40 · see citing works
The Uncommon Phenomenon of Short QT Syndrome: A Scoping Review of the Literature
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Prevalence, prognosis and characteristics of subjects with short QT interval in an electrocardiogram
Anttonen, O. M. (2009) · Cited by 7 · see citing works
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Maury, P., Extramiana, F., Giustetto, C., Cardin, C., Rollin, A., Duparc, A., Mondoly, P., Denjoy, I., Delay, M., MESSALI, A., et al. (2012) · Pacing and Clinical Electrophysiology · doi:10.1111/j.1540-8159.2012.03491.x · Cited by 6 · see citing works
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Okishige, K., Sugiyama, K., Maeda, M., Aoyagi, H., Kurabayashi, M., Miyagi, N., Ueshima, D., Azegami, K., Takei, T., Itoh, T., et al. (2009) · Journal of Arrhythmia · doi:10.1016/s1880-4276(09)80006-9 · Cited by 1 · see citing works
Acquired (Drug-Induced) Long and Short QT Syndromes
Shah, R., & Gussak, I. (2013) · Springer eBooks · doi:10.1007/978-1-4471-4978-1_6 · Cited by 2 · see citing works
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Stern, S. (2010) · Annals of Noninvasive Electrocardiology · doi:10.1111/j.1542-474x.2009.00347.x · Cited by 1 · see citing works
Extramiana, F., Tuan, T.-V. N. M., Vaglio, M., Ozguler, A., Macfarlane, P. W., Maison‐Blanche, P., & Badilini, F. (2026) · Archives of cardiovascular diseases · doi:10.1016/j.acvd.2025.12.013
Self-citation
- Authors
- Extramiana, F., Maury, P., Maison-Blanche, P., Duparc, A., Delay, M., & Leenhardt, A.
- Affiliations
- Assistance Publique – Hôpitaux de Paris (FR)
- Hôpital Rangueil (FR)
- Corresponding author
- Extramiana, Fabrice
- Venue
- The American Journal of Cardiology
- Publisher
- Elsevier BV
- Published
- 2008-03
- Volume
- 101
- Issue
- 6
- Pages
- 855-860
- ISSN
- 0002-9149 (ISSN-L)
- OpenAlex
- W2067588508
- Cited by
- 15
- References
- 28
- Citations by year
- 2026: 1 · 2025: 1 · 2013: 1 · 2012: 1
- FWCI
- 2.67
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The American Journal of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac pacing and defibrillation studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Keywords
- short QT syndrome, ventricular repolarization, QT interval correction, QT/RR relationship, Cronbach's alpha, Fridericia formula, Hodges formula, Holter monitoring, family studies, T wave changes
- MeSH terms
- Adolescent, Adult, Algorithms, Bradycardia, Diagnosis, Differential, Electrocardiography, Family, Female, Follow-Up Studies, Heart Rate, Humans, Male, Models, Theoretical, Pedigree, Prognosis, Genetic Predisposition to Disease
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