Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy

Carla Spazzolini, Jamie Mullally, Arthur J. Moss, Peter J. Schwartz, Scott McNitt, Gregory Ouellet, Thomas Fugate, Ilan Goldenberg, Christian Jons, Wojciech Zareba, Jennifer L. Robinson, Michael J. Ackerman, Jesaia Benhorin, Lia Crotti, Elizabeth S. Kaufman, Emanuela H. Locati, Ming Qi, Carlo Napolitano, Silvia G. Priori, Jeffrey Towbin & 1 others G. Michael Vincent

Research output: Contribution to journalArticle

53 Citations (Scopus)

Abstract

Objectives: This study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy). Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously. Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) ≥450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life. Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc ≥500 ms, heart rate ≤100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p < 0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy. Conclusions: Patients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset.

Original languageEnglish (US)
Pages (from-to)832-837
Number of pages6
JournalJournal of the American College of Cardiology
Volume54
Issue number9
DOIs
StatePublished - Aug 25 2009
Externally publishedYes

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Long QT Syndrome
Heart Arrest
Sudden Cardiac Death
Syncope
Heart Rate
Registries
Electrocardiography

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Spazzolini, C., Mullally, J., Moss, A. J., Schwartz, P. J., McNitt, S., Ouellet, G., ... Vincent, G. M. (2009). Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy. Journal of the American College of Cardiology, 54(9), 832-837. https://doi.org/10.1016/j.jacc.2009.05.029

Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy. / Spazzolini, Carla; Mullally, Jamie; Moss, Arthur J.; Schwartz, Peter J.; McNitt, Scott; Ouellet, Gregory; Fugate, Thomas; Goldenberg, Ilan; Jons, Christian; Zareba, Wojciech; Robinson, Jennifer L.; Ackerman, Michael J.; Benhorin, Jesaia; Crotti, Lia; Kaufman, Elizabeth S.; Locati, Emanuela H.; Qi, Ming; Napolitano, Carlo; Priori, Silvia G.; Towbin, Jeffrey; Vincent, G. Michael.

In: Journal of the American College of Cardiology, Vol. 54, No. 9, 25.08.2009, p. 832-837.

Research output: Contribution to journalArticle

Spazzolini, C, Mullally, J, Moss, AJ, Schwartz, PJ, McNitt, S, Ouellet, G, Fugate, T, Goldenberg, I, Jons, C, Zareba, W, Robinson, JL, Ackerman, MJ, Benhorin, J, Crotti, L, Kaufman, ES, Locati, EH, Qi, M, Napolitano, C, Priori, SG, Towbin, J & Vincent, GM 2009, 'Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy', Journal of the American College of Cardiology, vol. 54, no. 9, pp. 832-837. https://doi.org/10.1016/j.jacc.2009.05.029
Spazzolini, Carla ; Mullally, Jamie ; Moss, Arthur J. ; Schwartz, Peter J. ; McNitt, Scott ; Ouellet, Gregory ; Fugate, Thomas ; Goldenberg, Ilan ; Jons, Christian ; Zareba, Wojciech ; Robinson, Jennifer L. ; Ackerman, Michael J. ; Benhorin, Jesaia ; Crotti, Lia ; Kaufman, Elizabeth S. ; Locati, Emanuela H. ; Qi, Ming ; Napolitano, Carlo ; Priori, Silvia G. ; Towbin, Jeffrey ; Vincent, G. Michael. / Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy. In: Journal of the American College of Cardiology. 2009 ; Vol. 54, No. 9. pp. 832-837.
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title = "Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy",
abstract = "Objectives: This study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy). Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously. Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) ≥450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life. Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc ≥500 ms, heart rate ≤100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p < 0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy. Conclusions: Patients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset.",
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T1 - Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy

AU - Spazzolini, Carla

AU - Mullally, Jamie

AU - Moss, Arthur J.

AU - Schwartz, Peter J.

AU - McNitt, Scott

AU - Ouellet, Gregory

AU - Fugate, Thomas

AU - Goldenberg, Ilan

AU - Jons, Christian

AU - Zareba, Wojciech

AU - Robinson, Jennifer L.

AU - Ackerman, Michael J.

AU - Benhorin, Jesaia

AU - Crotti, Lia

AU - Kaufman, Elizabeth S.

AU - Locati, Emanuela H.

AU - Qi, Ming

AU - Napolitano, Carlo

AU - Priori, Silvia G.

AU - Towbin, Jeffrey

AU - Vincent, G. Michael

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N2 - Objectives: This study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy). Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously. Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) ≥450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life. Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc ≥500 ms, heart rate ≤100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p < 0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy. Conclusions: Patients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset.

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