Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2

Jonathan Buber, Jehu Mathew, Arthur J. Moss, W. Jackson Hall, Alon Barsheshet, Scott McNitt, Jennifer L. Robinson, Wojciech Zareba, Michael John Ackerman, Elizabeth S. Kaufman, David Luria, Michael Eldar, Jeffrey A. Towbin, Michael Vincent, Ilan Goldenberg

Research output: Contribution to journalArticle

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Abstract

Background-: Women with congenital long-QT syndrome experience an increased risk for cardiac events after the onset of adolescence that is more pronounced among carriers of the LQT2 genotype. We hypothesized that the hormonal changes associated with menopause may affect clinical risk in this population. Methods and Results-: We used a repeated-events analysis to evaluate the risk for recurrent syncope during the menopause transition and postmenopausal periods (5 years before and after the age at onset of menopause, respectively) among 282 LQT1 (n=151) and LQT2 (n=131) women enrolled in the Long-QT Syndrome Registry. Multivariate analysis showed that the risk for recurrent syncope (n=150) among LQT2 women was significantly increased during both menopause transition (hazard ratio, 3.38; P=0.005) and the postmenopausal period (hazard ratio, 8.10; P<0.001) compared with the reproductive period. The risk increase was evident among women who did or did not receive estrogen therapy. In contrast, among LQT1 women, the onset of menopause was associated with a reduction in the risk for recurrent syncope (hazard ratio, 0.19; P=0.05; P=0.02 for genotype-by-menopause interaction). Only 22 women (8%) experienced aborted cardiac arrest or sudden cardiac death during follow-up. The frequency of aborted cardiac arrest/sudden cardiac death showed a similar genotype-specific association with the onset of menopause. Conclusions-: The onset of menopause is associated with a significant increase in the risk of cardiac events (dominated by recurrent episodes of syncope) in LQT2 women, suggesting that careful follow-up and continued long-term therapy are warranted in this population.

Original languageEnglish (US)
Pages (from-to)2784-2791
Number of pages8
JournalCirculation
Volume123
Issue number24
DOIs
StatePublished - Jun 21 2011

Fingerprint

Romano-Ward Syndrome
Menopause
Syncope
Postmenopause
Long QT Syndrome
Sudden Cardiac Death
Genotype
Heart Arrest
Risk Reduction Behavior
Age of Onset
Population
Reproduction
Registries
Estrogens
Multivariate Analysis

Keywords

  • estrogen
  • long QT syndrome
  • testosterone
  • women

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Buber, J., Mathew, J., Moss, A. J., Hall, W. J., Barsheshet, A., McNitt, S., ... Goldenberg, I. (2011). Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2. Circulation, 123(24), 2784-2791. https://doi.org/10.1161/CIRCULATIONAHA.110.000620

Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2. / Buber, Jonathan; Mathew, Jehu; Moss, Arthur J.; Hall, W. Jackson; Barsheshet, Alon; McNitt, Scott; Robinson, Jennifer L.; Zareba, Wojciech; Ackerman, Michael John; Kaufman, Elizabeth S.; Luria, David; Eldar, Michael; Towbin, Jeffrey A.; Vincent, Michael; Goldenberg, Ilan.

In: Circulation, Vol. 123, No. 24, 21.06.2011, p. 2784-2791.

Research output: Contribution to journalArticle

Buber, J, Mathew, J, Moss, AJ, Hall, WJ, Barsheshet, A, McNitt, S, Robinson, JL, Zareba, W, Ackerman, MJ, Kaufman, ES, Luria, D, Eldar, M, Towbin, JA, Vincent, M & Goldenberg, I 2011, 'Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2', Circulation, vol. 123, no. 24, pp. 2784-2791. https://doi.org/10.1161/CIRCULATIONAHA.110.000620
Buber, Jonathan ; Mathew, Jehu ; Moss, Arthur J. ; Hall, W. Jackson ; Barsheshet, Alon ; McNitt, Scott ; Robinson, Jennifer L. ; Zareba, Wojciech ; Ackerman, Michael John ; Kaufman, Elizabeth S. ; Luria, David ; Eldar, Michael ; Towbin, Jeffrey A. ; Vincent, Michael ; Goldenberg, Ilan. / Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2. In: Circulation. 2011 ; Vol. 123, No. 24. pp. 2784-2791.
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abstract = "Background-: Women with congenital long-QT syndrome experience an increased risk for cardiac events after the onset of adolescence that is more pronounced among carriers of the LQT2 genotype. We hypothesized that the hormonal changes associated with menopause may affect clinical risk in this population. Methods and Results-: We used a repeated-events analysis to evaluate the risk for recurrent syncope during the menopause transition and postmenopausal periods (5 years before and after the age at onset of menopause, respectively) among 282 LQT1 (n=151) and LQT2 (n=131) women enrolled in the Long-QT Syndrome Registry. Multivariate analysis showed that the risk for recurrent syncope (n=150) among LQT2 women was significantly increased during both menopause transition (hazard ratio, 3.38; P=0.005) and the postmenopausal period (hazard ratio, 8.10; P<0.001) compared with the reproductive period. The risk increase was evident among women who did or did not receive estrogen therapy. In contrast, among LQT1 women, the onset of menopause was associated with a reduction in the risk for recurrent syncope (hazard ratio, 0.19; P=0.05; P=0.02 for genotype-by-menopause interaction). Only 22 women (8{\%}) experienced aborted cardiac arrest or sudden cardiac death during follow-up. The frequency of aborted cardiac arrest/sudden cardiac death showed a similar genotype-specific association with the onset of menopause. Conclusions-: The onset of menopause is associated with a significant increase in the risk of cardiac events (dominated by recurrent episodes of syncope) in LQT2 women, suggesting that careful follow-up and continued long-term therapy are warranted in this population.",
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AU - McNitt, Scott

AU - Robinson, Jennifer L.

AU - Zareba, Wojciech

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