Published online May 26, 2008
PEDIATRICS (doi:10.1542/peds.2007-2807)
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ARTICLE

Turner Syndrome Is an Independent Risk Factor for Aortic Dilation in the Young

Leo Lopez, MDa, Kristopher L. Arheart, EdDb, Steven D. Colan, MDc, Nancy S. Stein, PhD, MPHd, Gabriela Lopez-Mitnik, MSd, Angela E. Lin, MDe, Mark D. Reller, MDf, Roque Ventura, RCSa and Michael Silberbach, MDf

a Congenital Heart Institute, Miami Children's Hospital, Miami, Florida; Departments of
b Epidemiology and Public Health
d Pediatrics, University of Miami, Miami, Florida
c Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts
e Genetics Unit, Massachusetts General Hospital for Children, Boston, Massachusetts
f Division of Pediatric Cardiology, Doernbecher Children's Hospital, Oregon Health and Science University, Portland, Oregon

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.

Key Words: Turner syndrome • aortic dilation • aortic dissection • aortic z scores • bicuspid aortic valve

Abbreviations: BAV—bicuspid aortic valve • GH—growth hormone • BP—blood pressure • BSA—body surface area • ANN—aortic annulus • AR—aortic root • STJ—sinotubular junction • AAO—ascending aorta • DTA—distal transverse aortic arch • IST—aortic isthmus

Accepted Nov 19, 2007.