Dissection of the Aorta in Turner Syndrome: Two new cases and review of 85 cases in the literature

Misty Carlson ¹ and Michael Silberbach ^1*

¹ Oregon Health & Science University, United States

^* To whom correspondence should be addressed. E-mail: silberbm{at}ohsu.edu.

Accepted 16 August 2007

Abstract

Background: 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. Methods: In addition to reporting 2 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. Results: Eighty-five cases of aortic dissection in TS have been reported between 1961 and the present. Dissection occurred at a young age, 30.7 (range 4-64) years, which is significantly earlier than its occurrence in the general female population (68y). Of the cases where HTN and CHD were explicitly assessed, 15% had HTN alone, 30% had only CHD, 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. The cases where no risk factors were identified were very poorly documented, however. Dissection in TS women undergoing assisted reproductive techniques (ART) frequently results in death. Conclusion: The literature of aortic dissection in TS is sparse and most cases are poorly documented making it very difficult to establish guidelines regarding monitoring and treatment. A TS aortic dissection registry has been established to determine the natural history and risk factors (http://www.tssus.org/readweb.asp?wid=3092).

Keywords: Aortic aneurysm, Aortic dissection, Congenital heart disease, Sudden death, Turner syndrome

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