Effectiveness and limitations of β-blocker therapy in congenital long- QT syndrome

Arthur J. Moss, Wojciech Zareba, W. Jackson Hall, Peter J. Schwartz, Richard S. Crampton, Jesaia Benhorin, G. Michael Vincent, Emanuela H. Locati, Silvia G. Priori, Carlo Napolitano, Aharon Medina, Li Zhang, Jennifer L. Robinson, Katherine Timothy, Jeffrey Towbin, Mark L. Andrews

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Abstract

Background - β-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness ant limitations of β-blockers in this disorder have not been evaluated. Methods and Results - The study population comprised 869 LQTS patients treated with β-blockers. Effectiveness of β- blockers was analyzed during matched periods before and after starting β- blocker therapy, and by survivorship methods to determine factors associated with cardiac events while on prescribed β-blockers. After initiation of β- blockers, there was a significant (P<0.001) reduction in the rate of cardiac events in probands (0.97±1.42 to 0.31±0.86 events per year) and in affected family member (0.26±0.84 to 0.15±0.69 events per year) during 5-year matched periods. On-therapy survivorship analyses revealed that patient: with cardiac symptoms before β-blockers (n=598) had a hazard ratio of 5.8 (95% CI, 3.7 to 9.1) for recurrent cardiac events (syncope, aborted cardiac arrest, or death) during β-blocker therapy compared with asymptomatic patients; 32% of these symptomatic patients will have another cardiac event within 5 years while on prescribed β-blockers. Patients with a history of aborted cardiac arrest before starting β-blockers (n=113) had a hazard ratio of 12.9 (95% CI, 4.7 to 35.5) for aborted cardiac arrest or death while on prescribed β-blockers compared with asymptomatic patients; 14% of these patients will have another arrest (aborted or fatal) within 5 years on β- blockers. Conclusionsβ-blockers are associated with a significant reduction in cardiac events in LQTS patients. However, syncope, aborted cardiac arrest, and LQTS-related death continue to occur while patients are on prescribed β- blockers, particularly in those who were symptomatic before starting this therapy.

Original languageEnglish (US)
Pages (from-to)616-623
Number of pages8
JournalCirculation
Volume101
Issue number6
DOIs
StatePublished - Feb 15 2000

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Long QT Syndrome
Heart Arrest
Therapeutics
Syncope
Survival Rate
Ants

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Moss, A. J., Zareba, W., Hall, W. J., Schwartz, P. J., Crampton, R. S., Benhorin, J., ... Andrews, M. L. (2000). Effectiveness and limitations of β-blocker therapy in congenital long- QT syndrome. Circulation, 101(6), 616-623. https://doi.org/10.1161/01.CIR.101.6.616

Effectiveness and limitations of β-blocker therapy in congenital long- QT syndrome. / Moss, Arthur J.; Zareba, Wojciech; Hall, W. Jackson; Schwartz, Peter J.; Crampton, Richard S.; Benhorin, Jesaia; Vincent, G. Michael; Locati, Emanuela H.; Priori, Silvia G.; Napolitano, Carlo; Medina, Aharon; Zhang, Li; Robinson, Jennifer L.; Timothy, Katherine; Towbin, Jeffrey; Andrews, Mark L.

In: Circulation, Vol. 101, No. 6, 15.02.2000, p. 616-623.

Research output: Contribution to journalArticle

Moss, AJ, Zareba, W, Hall, WJ, Schwartz, PJ, Crampton, RS, Benhorin, J, Vincent, GM, Locati, EH, Priori, SG, Napolitano, C, Medina, A, Zhang, L, Robinson, JL, Timothy, K, Towbin, J & Andrews, ML 2000, 'Effectiveness and limitations of β-blocker therapy in congenital long- QT syndrome', Circulation, vol. 101, no. 6, pp. 616-623. https://doi.org/10.1161/01.CIR.101.6.616
Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J et al. Effectiveness and limitations of β-blocker therapy in congenital long- QT syndrome. Circulation. 2000 Feb 15;101(6):616-623. https://doi.org/10.1161/01.CIR.101.6.616
Moss, Arthur J. ; Zareba, Wojciech ; Hall, W. Jackson ; Schwartz, Peter J. ; Crampton, Richard S. ; Benhorin, Jesaia ; Vincent, G. Michael ; Locati, Emanuela H. ; Priori, Silvia G. ; Napolitano, Carlo ; Medina, Aharon ; Zhang, Li ; Robinson, Jennifer L. ; Timothy, Katherine ; Towbin, Jeffrey ; Andrews, Mark L. / Effectiveness and limitations of β-blocker therapy in congenital long- QT syndrome. In: Circulation. 2000 ; Vol. 101, No. 6. pp. 616-623.
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abstract = "Background - β-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness ant limitations of β-blockers in this disorder have not been evaluated. Methods and Results - The study population comprised 869 LQTS patients treated with β-blockers. Effectiveness of β- blockers was analyzed during matched periods before and after starting β- blocker therapy, and by survivorship methods to determine factors associated with cardiac events while on prescribed β-blockers. After initiation of β- blockers, there was a significant (P<0.001) reduction in the rate of cardiac events in probands (0.97±1.42 to 0.31±0.86 events per year) and in affected family member (0.26±0.84 to 0.15±0.69 events per year) during 5-year matched periods. On-therapy survivorship analyses revealed that patient: with cardiac symptoms before β-blockers (n=598) had a hazard ratio of 5.8 (95{\%} CI, 3.7 to 9.1) for recurrent cardiac events (syncope, aborted cardiac arrest, or death) during β-blocker therapy compared with asymptomatic patients; 32{\%} of these symptomatic patients will have another cardiac event within 5 years while on prescribed β-blockers. Patients with a history of aborted cardiac arrest before starting β-blockers (n=113) had a hazard ratio of 12.9 (95{\%} CI, 4.7 to 35.5) for aborted cardiac arrest or death while on prescribed β-blockers compared with asymptomatic patients; 14{\%} of these patients will have another arrest (aborted or fatal) within 5 years on β- blockers. Conclusionsβ-blockers are associated with a significant reduction in cardiac events in LQTS patients. However, syncope, aborted cardiac arrest, and LQTS-related death continue to occur while patients are on prescribed β- blockers, particularly in those who were symptomatic before starting this therapy.",
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T1 - Effectiveness and limitations of β-blocker therapy in congenital long- QT syndrome

AU - Moss, Arthur J.

AU - Zareba, Wojciech

AU - Hall, W. Jackson

AU - Schwartz, Peter J.

AU - Crampton, Richard S.

AU - Benhorin, Jesaia

AU - Vincent, G. Michael

AU - Locati, Emanuela H.

AU - Priori, Silvia G.

AU - Napolitano, Carlo

AU - Medina, Aharon

AU - Zhang, Li

AU - Robinson, Jennifer L.

AU - Timothy, Katherine

AU - Towbin, Jeffrey

AU - Andrews, Mark L.

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N2 - Background - β-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness ant limitations of β-blockers in this disorder have not been evaluated. Methods and Results - The study population comprised 869 LQTS patients treated with β-blockers. Effectiveness of β- blockers was analyzed during matched periods before and after starting β- blocker therapy, and by survivorship methods to determine factors associated with cardiac events while on prescribed β-blockers. After initiation of β- blockers, there was a significant (P<0.001) reduction in the rate of cardiac events in probands (0.97±1.42 to 0.31±0.86 events per year) and in affected family member (0.26±0.84 to 0.15±0.69 events per year) during 5-year matched periods. On-therapy survivorship analyses revealed that patient: with cardiac symptoms before β-blockers (n=598) had a hazard ratio of 5.8 (95% CI, 3.7 to 9.1) for recurrent cardiac events (syncope, aborted cardiac arrest, or death) during β-blocker therapy compared with asymptomatic patients; 32% of these symptomatic patients will have another cardiac event within 5 years while on prescribed β-blockers. Patients with a history of aborted cardiac arrest before starting β-blockers (n=113) had a hazard ratio of 12.9 (95% CI, 4.7 to 35.5) for aborted cardiac arrest or death while on prescribed β-blockers compared with asymptomatic patients; 14% of these patients will have another arrest (aborted or fatal) within 5 years on β- blockers. Conclusionsβ-blockers are associated with a significant reduction in cardiac events in LQTS patients. However, syncope, aborted cardiac arrest, and LQTS-related death continue to occur while patients are on prescribed β- blockers, particularly in those who were symptomatic before starting this therapy.

AB - Background - β-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness ant limitations of β-blockers in this disorder have not been evaluated. Methods and Results - The study population comprised 869 LQTS patients treated with β-blockers. Effectiveness of β- blockers was analyzed during matched periods before and after starting β- blocker therapy, and by survivorship methods to determine factors associated with cardiac events while on prescribed β-blockers. After initiation of β- blockers, there was a significant (P<0.001) reduction in the rate of cardiac events in probands (0.97±1.42 to 0.31±0.86 events per year) and in affected family member (0.26±0.84 to 0.15±0.69 events per year) during 5-year matched periods. On-therapy survivorship analyses revealed that patient: with cardiac symptoms before β-blockers (n=598) had a hazard ratio of 5.8 (95% CI, 3.7 to 9.1) for recurrent cardiac events (syncope, aborted cardiac arrest, or death) during β-blocker therapy compared with asymptomatic patients; 32% of these symptomatic patients will have another cardiac event within 5 years while on prescribed β-blockers. Patients with a history of aborted cardiac arrest before starting β-blockers (n=113) had a hazard ratio of 12.9 (95% CI, 4.7 to 35.5) for aborted cardiac arrest or death while on prescribed β-blockers compared with asymptomatic patients; 14% of these patients will have another arrest (aborted or fatal) within 5 years on β- blockers. Conclusionsβ-blockers are associated with a significant reduction in cardiac events in LQTS patients. However, syncope, aborted cardiac arrest, and LQTS-related death continue to occur while patients are on prescribed β- blockers, particularly in those who were symptomatic before starting this therapy.

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