title: Turner syndrome is an independent risk factor for aortic dilation in the young.
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.
  
  
  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.
authors.affiliation: Children's Hospital at Montefiore, Division of Pediatric Cardiology, 3415 Bainbridge Ave, Rosenthal 3, Bronx, NY 10467, USA. llmd@llmd.net
authors.forename: Leo
authors.initials: L
authors.lastname: Lopez
authors.forename: Kristopher L
authors.initials: KL
authors.lastname: Arheart
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Nancy S
authors.initials: NS
authors.lastname: Stein
authors.forename: Gabriela
authors.initials: G
authors.lastname: Lopez-Mitnik
authors.forename: Angela E
authors.initials: AE
authors.lastname: Lin
authors.forename: Mark D
authors.initials: MD
authors.lastname: Reller
authors.forename: Roque
authors.initials: R
authors.lastname: Ventura
authors.forename: Michael
authors.initials: M
authors.lastname: Silberbach
mesh_terms: D000293:Adolescent; D001018:Aortic Diseases / Q000209:etiology*; D002648:Child; D004108:Dilatation, Pathologic / Q000209:etiology; D005260:Female; D006801:Humans; D012307:Risk Factors; D014424:Turner Syndrome / Q000150:complications*