Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital Long-QT syndrome

Ilan Goldenberg, Arthur J. Moss, Derick R. Peterson, Scott McNitt, Wojciech Zareba, Mark L. Andrews, Jennifer L. Robinson, Emanuela H. Locati, Michael John Ackerman, Jesaia Benhorin, Elizabeth S. Kaufman, Carlo Napolitano, Silvia G. Priori, Ming Qi, Peter J. Schwartz, Jeffrey A. Towbin, G. Michael Vincent, Li Zhang

Research output: Contribution to journalArticle

200 Citations (Scopus)

Abstract

Background-The congenital long-QT syndrome (LQTS) is an important cause of sudden cardiac death in children without structural heart disease. However, specific risk factors for life-threatening cardiac events in children with this genetic disorder have not been identified. Methods and Results-Cox proportional-hazards regression modeling was used to identify risk factors for aborted cardiac arrest or sudden cardiac death in 3015 LQTS children from the International LQTS Registry who were followed up from 1 through 12 years of age. The cumulative probability of the combined end point was significantly higher in boys (5%) than in girls (1%; P<0.001). Risk factors for cardiac arrest or sudden cardiac death during childhood included corrected QT interval [QTc] duration >500 ms (hazard ratio [HR]; 2.72; 95% confidence interval [CI], 1.50 to 4.92; P=0.001) and prior syncope (recent syncope [<2 years]: HR, 6.16; 95% CI 3.41 to 11.15; P<0.001; remote syncope [≥2 years]: HR, 2.67; 95% CI, 1.22 to 5.85; P=0.01) in boys, whereas prior syncope was the only significant risk factor among girls (recent syncope: HR, 27.82; 95% CI, 9.72 to 79.60; P≤0.001; remote syncope: HR, 12.04; 95% CI, 3.79 to 38.26; P≤0.001). β-Blocker therapy was associated with a significant 53% reduction in the risk of cardiac arrest or sudden cardiac death (P=0.01). Conclusions-LQTS boys experience a significantly higher rate of fatal or near-fatal cardiac events than girls during childhood. A QTc duration ≥500 ms and a history of prior syncope identify risk in boys, whereas prior syncope is the only significant risk factor among girls. β-Blocker therapy is associated with a significant reduction in the risk of life-threatening cardiac events during childhood. (Circulation. 2008;117:2184-2191.).

Original languageEnglish (US)
Pages (from-to)2184-2191
Number of pages8
JournalCirculation
Volume117
Issue number17
DOIs
StatePublished - Apr 29 2008

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Long QT Syndrome
Sudden Cardiac Death
Syncope
Heart Arrest
Confidence Intervals
Risk Reduction Behavior
Inborn Genetic Diseases
Registries
Heart Diseases
Therapeutics

Keywords

  • Death
  • Long-QT syndrome
  • Risk factors
  • Sudden

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine
  • Physiology

Cite this

Goldenberg, I., Moss, A. J., Peterson, D. R., McNitt, S., Zareba, W., Andrews, M. L., ... Zhang, L. (2008). Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital Long-QT syndrome. Circulation, 117(17), 2184-2191. https://doi.org/10.1161/CIRCULATIONAHA.107.701243

Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital Long-QT syndrome. / Goldenberg, Ilan; Moss, Arthur J.; Peterson, Derick R.; McNitt, Scott; Zareba, Wojciech; Andrews, Mark L.; Robinson, Jennifer L.; Locati, Emanuela H.; Ackerman, Michael John; Benhorin, Jesaia; Kaufman, Elizabeth S.; Napolitano, Carlo; Priori, Silvia G.; Qi, Ming; Schwartz, Peter J.; Towbin, Jeffrey A.; Vincent, G. Michael; Zhang, Li.

In: Circulation, Vol. 117, No. 17, 29.04.2008, p. 2184-2191.

Research output: Contribution to journalArticle

Goldenberg, I, Moss, AJ, Peterson, DR, McNitt, S, Zareba, W, Andrews, ML, Robinson, JL, Locati, EH, Ackerman, MJ, Benhorin, J, Kaufman, ES, Napolitano, C, Priori, SG, Qi, M, Schwartz, PJ, Towbin, JA, Vincent, GM & Zhang, L 2008, 'Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital Long-QT syndrome', Circulation, vol. 117, no. 17, pp. 2184-2191. https://doi.org/10.1161/CIRCULATIONAHA.107.701243
Goldenberg, Ilan ; Moss, Arthur J. ; Peterson, Derick R. ; McNitt, Scott ; Zareba, Wojciech ; Andrews, Mark L. ; Robinson, Jennifer L. ; Locati, Emanuela H. ; Ackerman, Michael John ; Benhorin, Jesaia ; Kaufman, Elizabeth S. ; Napolitano, Carlo ; Priori, Silvia G. ; Qi, Ming ; Schwartz, Peter J. ; Towbin, Jeffrey A. ; Vincent, G. Michael ; Zhang, Li. / Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital Long-QT syndrome. In: Circulation. 2008 ; Vol. 117, No. 17. pp. 2184-2191.
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abstract = "Background-The congenital long-QT syndrome (LQTS) is an important cause of sudden cardiac death in children without structural heart disease. However, specific risk factors for life-threatening cardiac events in children with this genetic disorder have not been identified. Methods and Results-Cox proportional-hazards regression modeling was used to identify risk factors for aborted cardiac arrest or sudden cardiac death in 3015 LQTS children from the International LQTS Registry who were followed up from 1 through 12 years of age. The cumulative probability of the combined end point was significantly higher in boys (5{\%}) than in girls (1{\%}; P<0.001). Risk factors for cardiac arrest or sudden cardiac death during childhood included corrected QT interval [QTc] duration >500 ms (hazard ratio [HR]; 2.72; 95{\%} confidence interval [CI], 1.50 to 4.92; P=0.001) and prior syncope (recent syncope [<2 years]: HR, 6.16; 95{\%} CI 3.41 to 11.15; P<0.001; remote syncope [≥2 years]: HR, 2.67; 95{\%} CI, 1.22 to 5.85; P=0.01) in boys, whereas prior syncope was the only significant risk factor among girls (recent syncope: HR, 27.82; 95{\%} CI, 9.72 to 79.60; P≤0.001; remote syncope: HR, 12.04; 95{\%} CI, 3.79 to 38.26; P≤0.001). β-Blocker therapy was associated with a significant 53{\%} reduction in the risk of cardiac arrest or sudden cardiac death (P=0.01). Conclusions-LQTS boys experience a significantly higher rate of fatal or near-fatal cardiac events than girls during childhood. A QTc duration ≥500 ms and a history of prior syncope identify risk in boys, whereas prior syncope is the only significant risk factor among girls. β-Blocker therapy is associated with a significant reduction in the risk of life-threatening cardiac events during childhood. (Circulation. 2008;117:2184-2191.).",
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AU - Goldenberg, Ilan

AU - Moss, Arthur J.

AU - Peterson, Derick R.

AU - McNitt, Scott

AU - Zareba, Wojciech

AU - Andrews, Mark L.

AU - Robinson, Jennifer L.

AU - Locati, Emanuela H.

AU - Ackerman, Michael John

AU - Benhorin, Jesaia

AU - Kaufman, Elizabeth S.

AU - Napolitano, Carlo

AU - Priori, Silvia G.

AU - Qi, Ming

AU - Schwartz, Peter J.

AU - Towbin, Jeffrey A.

AU - Vincent, G. Michael

AU - Zhang, Li

PY - 2008/4/29

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N2 - Background-The congenital long-QT syndrome (LQTS) is an important cause of sudden cardiac death in children without structural heart disease. However, specific risk factors for life-threatening cardiac events in children with this genetic disorder have not been identified. Methods and Results-Cox proportional-hazards regression modeling was used to identify risk factors for aborted cardiac arrest or sudden cardiac death in 3015 LQTS children from the International LQTS Registry who were followed up from 1 through 12 years of age. The cumulative probability of the combined end point was significantly higher in boys (5%) than in girls (1%; P<0.001). Risk factors for cardiac arrest or sudden cardiac death during childhood included corrected QT interval [QTc] duration >500 ms (hazard ratio [HR]; 2.72; 95% confidence interval [CI], 1.50 to 4.92; P=0.001) and prior syncope (recent syncope [<2 years]: HR, 6.16; 95% CI 3.41 to 11.15; P<0.001; remote syncope [≥2 years]: HR, 2.67; 95% CI, 1.22 to 5.85; P=0.01) in boys, whereas prior syncope was the only significant risk factor among girls (recent syncope: HR, 27.82; 95% CI, 9.72 to 79.60; P≤0.001; remote syncope: HR, 12.04; 95% CI, 3.79 to 38.26; P≤0.001). β-Blocker therapy was associated with a significant 53% reduction in the risk of cardiac arrest or sudden cardiac death (P=0.01). Conclusions-LQTS boys experience a significantly higher rate of fatal or near-fatal cardiac events than girls during childhood. A QTc duration ≥500 ms and a history of prior syncope identify risk in boys, whereas prior syncope is the only significant risk factor among girls. β-Blocker therapy is associated with a significant reduction in the risk of life-threatening cardiac events during childhood. (Circulation. 2008;117:2184-2191.).

AB - Background-The congenital long-QT syndrome (LQTS) is an important cause of sudden cardiac death in children without structural heart disease. However, specific risk factors for life-threatening cardiac events in children with this genetic disorder have not been identified. Methods and Results-Cox proportional-hazards regression modeling was used to identify risk factors for aborted cardiac arrest or sudden cardiac death in 3015 LQTS children from the International LQTS Registry who were followed up from 1 through 12 years of age. The cumulative probability of the combined end point was significantly higher in boys (5%) than in girls (1%; P<0.001). Risk factors for cardiac arrest or sudden cardiac death during childhood included corrected QT interval [QTc] duration >500 ms (hazard ratio [HR]; 2.72; 95% confidence interval [CI], 1.50 to 4.92; P=0.001) and prior syncope (recent syncope [<2 years]: HR, 6.16; 95% CI 3.41 to 11.15; P<0.001; remote syncope [≥2 years]: HR, 2.67; 95% CI, 1.22 to 5.85; P=0.01) in boys, whereas prior syncope was the only significant risk factor among girls (recent syncope: HR, 27.82; 95% CI, 9.72 to 79.60; P≤0.001; remote syncope: HR, 12.04; 95% CI, 3.79 to 38.26; P≤0.001). β-Blocker therapy was associated with a significant 53% reduction in the risk of cardiac arrest or sudden cardiac death (P=0.01). Conclusions-LQTS boys experience a significantly higher rate of fatal or near-fatal cardiac events than girls during childhood. A QTc duration ≥500 ms and a history of prior syncope identify risk in boys, whereas prior syncope is the only significant risk factor among girls. β-Blocker therapy is associated with a significant reduction in the risk of life-threatening cardiac events during childhood. (Circulation. 2008;117:2184-2191.).

KW - Death

KW - Long-QT syndrome

KW - Risk factors

KW - Sudden

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