Clinical implications for affected parents and siblings of probands with long-QT syndrome

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

Research output: Contribution to journalArticle

60 Citations (Scopus)

Abstract

Background - Whenever a proband is identified with long-QT syndrome (LQTS), his or her parents and siblings should be evaluated regarding the possibility of carrying the disorder. In the majority of cases, one of the proband's parents and one or more siblings are affected. The aim of this study was (1) to determine whether the clinical severity of LQTS in the proband is useful in identifying first-degree family members at high risk for cardiac events, and (2) to evaluate the clinical course of affected parents and siblings of LQTS probands. Methods and Results - The clinical and ECG characteristics of 211 LQTS probands and 791 first-degree relatives (422 parents and 369 siblings) were studied to determine if the clinical profile of the proband is useful in determining the clinical severity of LQTS in affected parents and siblings. Affected female parents of an LQTS proband had a greater cumulative risk for a first cardiac event than affected male parents. The probability of a parent or sibling having a first cardiac event was not significantly influenced by the severity of the proband's clinical symptoms. Female sex and QTc duration were risk factors for cardiac events among affected parents, and QTc was the only risk factor for cardiac events in affected siblings. Conclusions - The severity profile of LQTS in a proband was not found to be useful in identifying the clinical severity of LQTS in affected first-degree relatives of the proband.

Original languageEnglish (US)
Pages (from-to)557-562
Number of pages6
JournalCirculation
Volume104
Issue number5
DOIs
StatePublished - Jul 31 2001
Externally publishedYes

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Long QT Syndrome
Siblings
Parents
Electrocardiography

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Kimbrough, J., Moss, A. J., Zareba, W., Robinson, J. L., Hall, W. J., Benhorin, J., ... Zhang, L. (2001). Clinical implications for affected parents and siblings of probands with long-QT syndrome. Circulation, 104(5), 557-562. https://doi.org/10.1161/hc3001.093501

Clinical implications for affected parents and siblings of probands with long-QT syndrome. / Kimbrough, John; Moss, Arthur J.; Zareba, Wojciech; Robinson, Jennifer L.; Hall, W. Jackson; Benhorin, Jesaia; Locati, Emanuela H.; Medina, Aharon; Napolitano, Carlo; Priori, Silvia; Schwartz, Peter J.; Timothy, Katherine; Towbin, Jeffrey; Vincent, G. Michael; Zhang, Li.

In: Circulation, Vol. 104, No. 5, 31.07.2001, p. 557-562.

Research output: Contribution to journalArticle

Kimbrough, J, Moss, AJ, Zareba, W, Robinson, JL, Hall, WJ, Benhorin, J, Locati, EH, Medina, A, Napolitano, C, Priori, S, Schwartz, PJ, Timothy, K, Towbin, J, Vincent, GM & Zhang, L 2001, 'Clinical implications for affected parents and siblings of probands with long-QT syndrome', Circulation, vol. 104, no. 5, pp. 557-562. https://doi.org/10.1161/hc3001.093501
Kimbrough J, Moss AJ, Zareba W, Robinson JL, Hall WJ, Benhorin J et al. Clinical implications for affected parents and siblings of probands with long-QT syndrome. Circulation. 2001 Jul 31;104(5):557-562. https://doi.org/10.1161/hc3001.093501
Kimbrough, John ; Moss, Arthur J. ; Zareba, Wojciech ; Robinson, Jennifer L. ; Hall, W. Jackson ; Benhorin, Jesaia ; Locati, Emanuela H. ; Medina, Aharon ; Napolitano, Carlo ; Priori, Silvia ; Schwartz, Peter J. ; Timothy, Katherine ; Towbin, Jeffrey ; Vincent, G. Michael ; Zhang, Li. / Clinical implications for affected parents and siblings of probands with long-QT syndrome. In: Circulation. 2001 ; Vol. 104, No. 5. pp. 557-562.
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AU - Moss, Arthur J.

AU - Zareba, Wojciech

AU - Robinson, Jennifer L.

AU - Hall, W. Jackson

AU - Benhorin, Jesaia

AU - Locati, Emanuela H.

AU - Medina, Aharon

AU - Napolitano, Carlo

AU - Priori, Silvia

AU - Schwartz, Peter J.

AU - Timothy, Katherine

AU - Towbin, Jeffrey

AU - Vincent, G. Michael

AU - Zhang, Li

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N2 - Background - Whenever a proband is identified with long-QT syndrome (LQTS), his or her parents and siblings should be evaluated regarding the possibility of carrying the disorder. In the majority of cases, one of the proband's parents and one or more siblings are affected. The aim of this study was (1) to determine whether the clinical severity of LQTS in the proband is useful in identifying first-degree family members at high risk for cardiac events, and (2) to evaluate the clinical course of affected parents and siblings of LQTS probands. Methods and Results - The clinical and ECG characteristics of 211 LQTS probands and 791 first-degree relatives (422 parents and 369 siblings) were studied to determine if the clinical profile of the proband is useful in determining the clinical severity of LQTS in affected parents and siblings. Affected female parents of an LQTS proband had a greater cumulative risk for a first cardiac event than affected male parents. The probability of a parent or sibling having a first cardiac event was not significantly influenced by the severity of the proband's clinical symptoms. Female sex and QTc duration were risk factors for cardiac events among affected parents, and QTc was the only risk factor for cardiac events in affected siblings. Conclusions - The severity profile of LQTS in a proband was not found to be useful in identifying the clinical severity of LQTS in affected first-degree relatives of the proband.

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