Turner syndrome is an independent risk factor for aortic dilation in the young

Leo Lopez, Kristopher Arheart, Steven D. Colan, Nancy S. Stein, Gabriela Lopez-Mitnik, Angela E. Lin, Mark D. Reller, Roque Ventura, Michael Silberbach

Research output: Contribution to journalArticle

74 Citations (Scopus)

Abstract

Objective. Because aortic dilation increases the risk for dissection in the general adult population, and dissection occurs with greater frequency at a young age with Turner syndrome, we studied the prevalence, magnitude, and determinants of aortic dilation in a large group of girls and young women with Turner syndrome. Patients and Methods. Participants at annual Turner syndrome society meetings completed a questionnaire regarding their medical history. Echocardiographic measurements of their aorta were converted to z scores by using data from a larger group of normal control female subjects. Bivariable and multivariable analyses evaluated the effects of Turner syndrome features, such as a bicuspid aortic valve, coarctation, growth-hormone therapy, blood pressure, and karyotype, on aortic size. Results. Among 138 individuals with Turner syndrome <18 years old, 49% had the 45,X karyotype, 26% had bicuspid aortic valve, 17% had a history of coarctation, 78% had a history of growth-hormone therapy, and 40% had hypertension. Aortic z scores were calculated by using data from 407 control subjects. Bivariable analyses revealed that a bicuspid aortic valve, growth hormone, and 45,X karyotype predicted a larger proximal aorta at ≥ 1 level. Multivariable analysis predicted a larger proximal aorta at all of the levels only for bicuspid aortic valve individuals and at the annular level for those who received growth hormone. Importantly, all of the analyses revealed that Turner syndrome predicted a larger proximal aorta independent of these characteristics. Conclusions. Among young individuals with Turner syndrome, a bicuspid aortic valve predicts a larger proximal aorta, and growth-hormone use may predict a larger aortic annulus. Compared with a control population, Turner syndrome alone is an independent risk factor for aortic dilation.

Original languageEnglish
JournalPediatrics
Volume121
Issue number6
DOIs
StatePublished - Jun 1 2008

Fingerprint

Turner Syndrome
Dilatation
Growth Hormone
Aorta
Karyotype
Dissection
Aortic Coarctation
Population
Bicuspid Aortic Valve
Blood Pressure
Hypertension
Control Groups
Therapeutics

Keywords

  • Aortic dilation
  • Aortic dissection
  • Aortic z scores
  • Bicuspid aortic valve
  • Turner syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Lopez, L., Arheart, K., Colan, S. D., Stein, N. S., Lopez-Mitnik, G., Lin, A. E., ... Silberbach, M. (2008). Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics, 121(6). https://doi.org/10.1542/peds.2007-2807

Turner syndrome is an independent risk factor for aortic dilation in the young. / Lopez, Leo; Arheart, Kristopher; Colan, Steven D.; Stein, Nancy S.; Lopez-Mitnik, Gabriela; Lin, Angela E.; Reller, Mark D.; Ventura, Roque; Silberbach, Michael.

In: Pediatrics, Vol. 121, No. 6, 01.06.2008.

Research output: Contribution to journalArticle

Lopez, L, Arheart, K, Colan, SD, Stein, NS, Lopez-Mitnik, G, Lin, AE, Reller, MD, Ventura, R & Silberbach, M 2008, 'Turner syndrome is an independent risk factor for aortic dilation in the young', Pediatrics, vol. 121, no. 6. https://doi.org/10.1542/peds.2007-2807
Lopez L, Arheart K, Colan SD, Stein NS, Lopez-Mitnik G, Lin AE et al. Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics. 2008 Jun 1;121(6). https://doi.org/10.1542/peds.2007-2807
Lopez, Leo ; Arheart, Kristopher ; Colan, Steven D. ; Stein, Nancy S. ; Lopez-Mitnik, Gabriela ; Lin, Angela E. ; Reller, Mark D. ; Ventura, Roque ; Silberbach, Michael. / Turner syndrome is an independent risk factor for aortic dilation in the young. In: Pediatrics. 2008 ; Vol. 121, No. 6.
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abstract = "Objective. Because aortic dilation increases the risk for dissection in the general adult population, and dissection occurs with greater frequency at a young age with Turner syndrome, we studied the prevalence, magnitude, and determinants of aortic dilation in a large group of girls and young women with Turner syndrome. Patients and Methods. Participants at annual Turner syndrome society meetings completed a questionnaire regarding their medical history. Echocardiographic measurements of their aorta were converted to z scores by using data from a larger group of normal control female subjects. Bivariable and multivariable analyses evaluated the effects of Turner syndrome features, such as a bicuspid aortic valve, coarctation, growth-hormone therapy, blood pressure, and karyotype, on aortic size. Results. Among 138 individuals with Turner syndrome <18 years old, 49{\%} had the 45,X karyotype, 26{\%} had bicuspid aortic valve, 17{\%} had a history of coarctation, 78{\%} had a history of growth-hormone therapy, and 40{\%} had hypertension. Aortic z scores were calculated by using data from 407 control subjects. Bivariable analyses revealed that a bicuspid aortic valve, growth hormone, and 45,X karyotype predicted a larger proximal aorta at ≥ 1 level. Multivariable analysis predicted a larger proximal aorta at all of the levels only for bicuspid aortic valve individuals and at the annular level for those who received growth hormone. Importantly, all of the analyses revealed that Turner syndrome predicted a larger proximal aorta independent of these characteristics. Conclusions. Among young individuals with Turner syndrome, a bicuspid aortic valve predicts a larger proximal aorta, and growth-hormone use may predict a larger aortic annulus. Compared with a control population, Turner syndrome alone is an independent risk factor for aortic dilation.",
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AU - Lopez, Leo

AU - Arheart, Kristopher

AU - Colan, Steven D.

AU - Stein, Nancy S.

AU - Lopez-Mitnik, Gabriela

AU - Lin, Angela E.

AU - Reller, Mark D.

AU - Ventura, Roque

AU - Silberbach, Michael

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N2 - Objective. Because aortic dilation increases the risk for dissection in the general adult population, and dissection occurs with greater frequency at a young age with Turner syndrome, we studied the prevalence, magnitude, and determinants of aortic dilation in a large group of girls and young women with Turner syndrome. Patients and Methods. Participants at annual Turner syndrome society meetings completed a questionnaire regarding their medical history. Echocardiographic measurements of their aorta were converted to z scores by using data from a larger group of normal control female subjects. Bivariable and multivariable analyses evaluated the effects of Turner syndrome features, such as a bicuspid aortic valve, coarctation, growth-hormone therapy, blood pressure, and karyotype, on aortic size. Results. Among 138 individuals with Turner syndrome <18 years old, 49% had the 45,X karyotype, 26% had bicuspid aortic valve, 17% had a history of coarctation, 78% had a history of growth-hormone therapy, and 40% had hypertension. Aortic z scores were calculated by using data from 407 control subjects. Bivariable analyses revealed that a bicuspid aortic valve, growth hormone, and 45,X karyotype predicted a larger proximal aorta at ≥ 1 level. Multivariable analysis predicted a larger proximal aorta at all of the levels only for bicuspid aortic valve individuals and at the annular level for those who received growth hormone. Importantly, all of the analyses revealed that Turner syndrome predicted a larger proximal aorta independent of these characteristics. Conclusions. Among young individuals with Turner syndrome, a bicuspid aortic valve predicts a larger proximal aorta, and growth-hormone use may predict a larger aortic annulus. Compared with a control population, Turner syndrome alone is an independent risk factor for aortic dilation.

AB - Objective. Because aortic dilation increases the risk for dissection in the general adult population, and dissection occurs with greater frequency at a young age with Turner syndrome, we studied the prevalence, magnitude, and determinants of aortic dilation in a large group of girls and young women with Turner syndrome. Patients and Methods. Participants at annual Turner syndrome society meetings completed a questionnaire regarding their medical history. Echocardiographic measurements of their aorta were converted to z scores by using data from a larger group of normal control female subjects. Bivariable and multivariable analyses evaluated the effects of Turner syndrome features, such as a bicuspid aortic valve, coarctation, growth-hormone therapy, blood pressure, and karyotype, on aortic size. Results. Among 138 individuals with Turner syndrome <18 years old, 49% had the 45,X karyotype, 26% had bicuspid aortic valve, 17% had a history of coarctation, 78% had a history of growth-hormone therapy, and 40% had hypertension. Aortic z scores were calculated by using data from 407 control subjects. Bivariable analyses revealed that a bicuspid aortic valve, growth hormone, and 45,X karyotype predicted a larger proximal aorta at ≥ 1 level. Multivariable analysis predicted a larger proximal aorta at all of the levels only for bicuspid aortic valve individuals and at the annular level for those who received growth hormone. Importantly, all of the analyses revealed that Turner syndrome predicted a larger proximal aorta independent of these characteristics. Conclusions. Among young individuals with Turner syndrome, a bicuspid aortic valve predicts a larger proximal aorta, and growth-hormone use may predict a larger aortic annulus. Compared with a control population, Turner syndrome alone is an independent risk factor for aortic dilation.

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KW - Aortic z scores

KW - Bicuspid aortic valve

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