The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Long QT Syndrome Patients With Mutations of the SCN5A and HERG Genes Have Differential Responses to Na + Channel Blockade and to Increases in Heart Rate: Implications for Gene-Specific Therapy
Abstract
Background The genes for the long QT syndrome (LQTS) linked to chromosomes 3 (LQT3) and 7 (LQT2) were identified as SCN5A , the cardiac Na + channel gene, and as HERG , a K + channel gene. These findings opened the possibility of attempting gene-specific control of ventricular repolarization. We tested the hypothesis that the QT interval would shorten more in LQT3 than in LQT2 patients in response to mexiletine and also in response to increases in heart rate. Methods and Results Fifteen LQTS patients were studied. Six LQT3 and 7 LQT2 patients were treated with mexiletine, and its effects on QT and QT c were measured. Mexiletine significantly shortened the QT interval among LQT3 patients (QT c from 535±32 to 445±31 ms, P <.005) but not among LQT2 patients (QT c from 530±79 to 503±60 ms, P =NS). LQT3 patients (n=7) shortened their QT interval in response to increases in heart rate much more than LQT2 patients (n=4) and also more than 18 healthy control subjects (9.45±3.3 versus 3.95±1.97 and 2.83±1.33, P <.05; data expressed as percent reduction in QT per 100-ms shortening in RR). Among these patients, there is also a trend for LQT2 patients to have syncope or cardiac arrest under emotional or physical stress and for LQT3 patients to have cardiac events either at rest or during sleep. Conclusions This is the first study to demonstrate differential responses of LQTS patients to interventions targeted to their specific genetic defect. These findings also suggest that LQT3 patients may be more likely to benefit from Na + channel blockers and from cardiac pacing because they would be at higher risk of arrhythmia at slow heart rates. Conversely, LQT2 patients may be at higher risk to develop syncope under stressful conditions because of the combined arrhythmogenic effect of catecholamines with the insufficient adaptation of their QT interval when heart rate increases.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
The long QT syndromes: A critical review, new clinical observations and a unifying hypothesis
Jackman, W. M., Friday, K. J., Anderson, J. L., Aliot, E. M., Clark, M., & Lazzara, R. (1988) · Progress in Cardiovascular Diseases · doi:10.1016/0033-0620(88)90014-x · Cited by 783
Cited by
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-citationPriori, 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
Self-citationSchwartz, 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
Self-citationBasic Mechanisms of Cardiac Impulse Propagation and Associated Arrhythmias
KLÉBER, A. G., & RUDY, Y. (2004) · Physiological Reviews · doi:10.1152/physrev.00025.2003 · Cited by 1,083
Maron, B. J., Chaitman, B. R., Ackerman, M. J., Bayés de Luna, A., Corrado, D., Crosson, J. E., Deal, B. J., Driscoll, D. J., Estes, N. A. M., Araújo, C. G. S., et al. (2004) · Circulation · doi:10.1161/01.cir.0000128363.85581.e1 · Cited by 591
Self-citation
Metadata
- Authors
- Schwartz, P. J., Priori, S. G., Locati, E. H., Napolitano, C., Cantù, F., Towbin, J. A., Keating, M. T., Hammoude, H., Brown, A. M., Chen, L.-S. K., & Colatsky, T. J.
- Venue
- Circulation
- Published
- 1995-12-15
- Volume
- 92
- Issue
- 12
- Pages
- 3381-3386
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2073709523
- Cited by
- 816
- References
- 1
- FWCI
- 30.71
- Type
- journal article
- Language
- en
- Open access
- closed
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
How to cite
Cite CoChat for the checks on this page, and the paper for its own findings.
- This page
- CoChat. (2026). Citation check: Long QT Syndrome Patients With Mutations of the SCN5A and HERG Genes Have Differential Responses to Na + Channel Blockade and to Increases in Heart Rate: Implications for Gene-Specific Therapy. https://app.cochat.ai/papers/10.1161/01.cir.92.12.3381
- The paper
- Schwartz, P. J., Priori, S. G., Locati, E. H., Napolitano, C., Cantù, F., Towbin, J. A., Keating, M. T., Hammoude, H., Brown, A. M., Chen, L.-S. K., & Colatsky, T. J. (1995). Long QT Syndrome Patients With Mutations of the SCN5A and HERG Genes Have Differential Responses to Na + Channel Blockade and to Increases in Heart Rate: Implications for Gene-Specific Therapy. Circulation. https://doi.org/10.1161/01.cir.92.12.3381
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
