The DOI matches its Crossref record; 27 of the 27 references it cites were found in a registry or an index.
High Efficacy of β-Blockers in Long-QT Syndrome Type 1: Contribution of Noncompliance and QT-Prolonging Drugs to the Occurrence of β-Blocker Treatment “Failures”
Abstract
Background— β-Blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite β-blocker therapy have not been ascertained. Methods and Results— This was a retrospective study of the details surrounding cardiac events in 216 genotyped long-QT syndrome type 1 patients treated with β-blocker and followed up for a median time of 10 years. Before β-blocker, cardiac events occurred in 157 patients (73%) at a median age of 9 years, with cardiac arrest (CA) in 26 (12%). QT-prolonging drugs were used by 17 patients; 9 of 17 (53%) had CA compared with 17 of 199 nonusers (8.5%; odds ratio, 12.0; 95% confidence interval, 4.1 to 35.3; P <0.001). After β-blocker, 75% were asymptomatic, and cardiac events were significantly reduced ( P <0.001), with a median event count (quartile 1 to 3) per person of 0 (0 to 1). Twelve patients (5.5%) suffered CA/sudden death, but 11 of 12 (92%) were noncompliant (n=8), were on a QT-prolonging drug (n=2), or both (n=1) at the time of the event. The risk for CA/sudden death in compliant patients not taking QT-prolonging drugs was dramatically less compared with noncompliant patients on QT-prolonging drugs (odds ratio, 0.03; 95% confidence interval, 0.003 to 0.22; P =0.001). None of the 26 patients with CA before β-blocker had CA/sudden death on β-blockers. Conclusions— β-Blockers are extremely effective in long-QT syndrome type 1 and should be administered at diagnosis and ideally before the preteen years. β-Blocker noncompliance and use of QT-prolonging drug are responsible for almost all life-threatening “β-blocker failures.” β-Blockers are appropriate therapy for asymptomatic patients and those who have never had a CA or β-blocker therapy. Routine implantation of cardiac defibrillators in such patients does not appear justified.
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Cites
A systematic review of the associations between dose regimens and medication compliance
Claxton, A. J., Cramer, J., & Pierce, C. (2001) · Clinical Therapeutics · doi:10.1016/s0149-2918(01)80109-0 · Cited by 2,388
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
Self-citationJervell, A., & Lange-Nielsen, F. (1957) · American Heart Journal · doi:10.1016/0002-8703(57)90079-0 · Cited by 1,596
Female Gender as a Risk Factor for Torsades de Pointes Associated With Cardiovascular Drugs
Makkar, R. R. (1993) · JAMA: The Journal of the American Medical Association · doi:10.1001/jama.1993.03510210076031 · Cited by 971
Schwartz, P. J., Periti, M., & Malliani, A. (1975) · American Heart Journal · doi:10.1016/0002-8703(75)90089-7 · Cited by 910
Self-citationEffectiveness and Limitations of β-Blocker Therapy in Congenital Long-QT Syndrome
Moss, A. J., Zareba, W., Hall, W. J., Schwartz, P. J., Crampton, R. S., Benhorin, J., Vincent, G. M., Locati, E. H., Priori, S. G., Napolitano, C., et al. (2000) · Circulation · doi:10.1161/01.cir.101.6.616 · Cited by 841
Self-citationThe Effect of Prescribed Daily Dose Frequency on Patient Medication Compliance
Eisen, S. A. (1990) · Archives of Internal Medicine · doi:10.1001/archinte.150.9.1881 · Cited by 817
Schwartz, P. J., Priori, S. G., Cerrone, M., Spazzolini, C., Odero, A., Napolitano, C., Bloise, R., De Ferrari, G. M., Klersy, C., Moss, A. J., et al. (2004) · Circulation · doi:10.1161/01.cir.0000125523.14403.1e · Cited by 686
Self-citationAssociation of Long QT Syndrome Loci and Cardiac Events Among Patients Treated With β-Blockers
Priori, S. G. (2004) · JAMA · doi:10.1001/jama.292.11.1341 · Cited by 601
Self-citationThe Jervell and Lange-Nielsen Syndrome: Natural History, Molecular Basis, and Clinical Outcome
Schwartz, P. J., Spazzolini, C., Crotti, L., Bathen, J., Amlie, J. P., Timothy, K., Shkolnikova, M., Berul, C. I., Bitner-Glindzicz, M., Toivonen, L., et al. (2006) · Circulation · doi:10.1161/circulationaha.105.592899 · Cited by 409
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2020 ESC guidelines on sports cardiology and exercise in patients with cardiovascular disease
Pelliccia, A., Sharma, S., Gati, S., Bäck, M., Börjesson, M., Caselli, S., Collet, J.-P., Corrado, D., Drezner, J. A., Halle, M., et al. (2021) · European Heart Journal · doi:10.1093/eurheartj/ehaa605 · Cited by 1,958
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
Self-citationPrevalence of the Congenital Long-QT Syndrome
Schwartz, P. J., Stramba-Badiale, M., Crotti, L., Pedrazzini, M., Besana, A., Bosi, G., Gabbarini, F., Goulene, K., Insolia, R., Mannarino, S., et al. (2009) · Circulation · doi:10.1161/circulationaha.109.863209 · Cited by 1,072
Self-citationAl-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) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2017.10.053 · Cited by 678
Long-QT Syndrome: From Genetics to Management
Schwartz, P. J., Crotti, L., & Insolia, R. (2012) · Circulation: Arrhythmia and Electrophysiology · doi:10.1161/circep.111.962019 · Cited by 659
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Metadata
- Authors
- Vincent, G. M., Schwartz, P. J., Denjoy, I., Swan, H., Bithell, C., Spazzolini, C., Crotti, L., Piippo, K., Lupoglazoff, J.-M., Villain, E., Priori, S. G., Napolitano, C., & Zhang, L.
- Venue
- Circulation
- Published
- 2009-01-20
- Volume
- 119
- Issue
- 2
- Pages
- 215-221
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2019045599
- Cited by
- 311
- References
- 27
- FWCI
- 17.40
- Type
- journal article
- Language
- en
- Open access
- closed
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- Vincent, G. M., Schwartz, P. J., Denjoy, I., Swan, H., Bithell, C., Spazzolini, C., Crotti, L., Piippo, K., Lupoglazoff, J.-M., Villain, E., Priori, S. G., Napolitano, C., & Zhang, L. (2009). High Efficacy of β-Blockers in Long-QT Syndrome Type 1: Contribution of Noncompliance and QT-Prolonging Drugs to the Occurrence of β-Blocker Treatment “Failures”. Circulation. https://doi.org/10.1161/circulationaha.108.772533
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