Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with the long QT syndrome

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

Research output: Contribution to journalArticle

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Abstract

In patients with the long QT syndrome (LQTS), the occurrence of cardiac events (syncope or cardiac arrest) is frequently associated with acute arousal caused by exercise, swimming, emotion, or noise. However, cardiac events may also occur during sleep or with ordinary daily activities. The purpose of this study was to determine whether there are differential clinical, electrocardiographic, and genetic features among LQTS patients who experienced cardiac events with and without acute arousal. We identified 1,325 patients with cardiac events from the International LQTS Registry. Based on the precipitating conditions of the first event, 427 patients were classified as arousal, 345 as nonarousal, and the remaining 553 were unknown (not classifiable). Gene linkage was known in 78 of the 772 patients with classifiable first events. The age at first cardiac event was significantly younger in the arousal than the nonarousal group (11.7 vs 15.5 years, respectively; p <0.001). The arousal-type patients had a higher rate of subsequent cardiac events during follow-up after the index event than the nonarousal-type patients (p = 0.02). Arousal-related cardiac events occurred in 85% of LQT1, 67% of LQT2, and 33% of LQT3 patients (p = 0.008). This study provides evidence that the genotype is an important determinant of the LQTS phenotype in terms of arousal and nonarousal-related cardiac events. (C) 2000 by Excerpta Medica, Inc.

Original languageEnglish (US)
Pages (from-to)457-461
Number of pages5
JournalAmerican Journal of Cardiology
Volume85
Issue number4
DOIs
StatePublished - Feb 15 2000
Externally publishedYes

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Long QT Syndrome
Arousal
Syncope
Heart Arrest
Registries
Noise
Sleep
Emotions
Genotype
Exercise
Phenotype

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with the long QT syndrome. / Ali, Raef H.Hajj; Zareba, Wojciech; Moss, Arthur J.; Schwartz, Peter J.; Benhorin, Jesaia; Vincent, G. Michael; Locati, Emanuela H.; Priori, Silvia; Napolitano, Carlo; Towbin, Jeffrey; Hall, W. Jackson; Robinson, Jennifer L.; Andrews, Mark L.; Zhang, Li; Timothy, Katherine; Medina, Aharon.

In: American Journal of Cardiology, Vol. 85, No. 4, 15.02.2000, p. 457-461.

Research output: Contribution to journalArticle

Ali, RHH, Zareba, W, Moss, AJ, Schwartz, PJ, Benhorin, J, Vincent, GM, Locati, EH, Priori, S, Napolitano, C, Towbin, J, Hall, WJ, Robinson, JL, Andrews, ML, Zhang, L, Timothy, K & Medina, A 2000, 'Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with the long QT syndrome', American Journal of Cardiology, vol. 85, no. 4, pp. 457-461. https://doi.org/10.1016/S0002-9149(99)90772-5
Ali, Raef H.Hajj ; Zareba, Wojciech ; Moss, Arthur J. ; Schwartz, Peter J. ; Benhorin, Jesaia ; Vincent, G. Michael ; Locati, Emanuela H. ; Priori, Silvia ; Napolitano, Carlo ; Towbin, Jeffrey ; Hall, W. Jackson ; Robinson, Jennifer L. ; Andrews, Mark L. ; Zhang, Li ; Timothy, Katherine ; Medina, Aharon. / Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with the long QT syndrome. In: American Journal of Cardiology. 2000 ; Vol. 85, No. 4. pp. 457-461.
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AU - Ali, Raef H.Hajj

AU - Zareba, Wojciech

AU - Moss, Arthur J.

AU - Schwartz, Peter J.

AU - Benhorin, Jesaia

AU - Vincent, G. Michael

AU - Locati, Emanuela H.

AU - Priori, Silvia

AU - Napolitano, Carlo

AU - Towbin, Jeffrey

AU - Hall, W. Jackson

AU - Robinson, Jennifer L.

AU - Andrews, Mark L.

AU - Zhang, Li

AU - Timothy, Katherine

AU - Medina, Aharon

PY - 2000/2/15

Y1 - 2000/2/15

N2 - In patients with the long QT syndrome (LQTS), the occurrence of cardiac events (syncope or cardiac arrest) is frequently associated with acute arousal caused by exercise, swimming, emotion, or noise. However, cardiac events may also occur during sleep or with ordinary daily activities. The purpose of this study was to determine whether there are differential clinical, electrocardiographic, and genetic features among LQTS patients who experienced cardiac events with and without acute arousal. We identified 1,325 patients with cardiac events from the International LQTS Registry. Based on the precipitating conditions of the first event, 427 patients were classified as arousal, 345 as nonarousal, and the remaining 553 were unknown (not classifiable). Gene linkage was known in 78 of the 772 patients with classifiable first events. The age at first cardiac event was significantly younger in the arousal than the nonarousal group (11.7 vs 15.5 years, respectively; p <0.001). The arousal-type patients had a higher rate of subsequent cardiac events during follow-up after the index event than the nonarousal-type patients (p = 0.02). Arousal-related cardiac events occurred in 85% of LQT1, 67% of LQT2, and 33% of LQT3 patients (p = 0.008). This study provides evidence that the genotype is an important determinant of the LQTS phenotype in terms of arousal and nonarousal-related cardiac events. (C) 2000 by Excerpta Medica, Inc.

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