HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS REGISTER		Author Keyword(s) Vol Page [Advanced]

This Article Full Text Full Text (PDF) web only tables All Versions of this Article: jmg.2007.052019v1 44/12/745    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Add article to my folders Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Carlson, M Articles by Silberbach, M Search for Related Content PubMed PubMed Citation Articles by Carlson, M Articles by Silberbach, M Topic Collections Editor's choice

Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature

Division of Pediatric Cardiology, Doernbecher Children’s Hospital, Oregon Health & Science University, Oregon, USA

Correspondence to:
M Silberbach, MD, Department of Pediatrics, CDRC-P, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, Portland, Oregon 97239-3098, USA; silberbm{at}ohsu.edu]

Girls and women with Turner syndrome are at risk for catastrophic aortic dissection and rupture, but the clinical profile for those at risk is not well described. In addition to reporting two new cases, we performed an electronic search to identify all reported cases of aortic dissection associated with Turner syndrome. Particular attention was paid to the reporting of systemic hypertension (HTN) and congenital heart disease (CHD) which are known risk factors for aortic disease in the general population. In total, 85 cases of aortic dissection in TS were reported between 1961 and 2006. Dissection occurred at a young age, 30.7 (range 4–64) years, which is significantly earlier than its occurrence in the general female population (68 years). Of the cases for which HTN and CHD were explicitly assessed, 15% had HTN alone, 30% had CHD alone and 34% had both. Importantly, in 11% of the cases, neither HTN nor CHD were identified, suggesting that TS alone is an independent risk factor for aortic dissection; however, the cases where no risk factors were identified were very poorly documented. Dissection in women with TS undergoing assisted reproductive techniques (ART) frequently resulted in death. The literature on aortic dissection in TS is sparse and most cases are poorly documented, making it difficult to establish firm guidelines regarding monitoring and treatment. A TS aortic dissection registry has been established to better determine the natural history and risk factors (http://www.tssus.org/readweb.asp?wid = 3092).

Abbreviations: ART, assisted reproductive techniques; BAV, bicuspid aortic valve; CHD, congenital heart disease, HTN, hypertension; TGF, transforming growth factor

This article has been cited by other articles:

				M. L. Loscalzo Turner Syndrome Pediatr. Rev., July 1, 2008; 29(7): 219 - 227. [Full Text] [PDF]

				L. Lopez, K. L. Arheart, S. D. Colan, N. S. Stein, G. Lopez-Mitnik, A. E. Lin, M. D. Reller, R. Ventura, and M. Silberbach Turner Syndrome Is an Independent Risk Factor for Aortic Dilation in the Young Pediatrics, June 1, 2008; 121(6): e1622 - e1627. [Abstract] [Full Text] [PDF]

				V. Sachdev, L. A. Matura, S. Sidenko, V. B. Ho, A. E. Arai, D. R. Rosing, and C. A. Bondy Aortic Valve Disease in Turner Syndrome J. Am. Coll. Cardiol., May 13, 2008; 51(19): 1904 - 1909. [Abstract] [Full Text] [PDF]