Aortic Dilatation and Dissection in Turner Syndrome
- 9 October 2007
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in Circulation
- Vol. 116 (15), 1663-1670
- https://doi.org/10.1161/circulationaha.106.685487
Abstract
Background— The risk for aortic dissection is increased among relatively young women with Turner syndrome (TS). It is unknown whether aortic dilatation precedes acute aortic dissection in TS and, if so, what specific diameter predicts impending deterioration. Methods and Results— Study subjects included 166 adult volunteers with TS (average age, 36.2 years) who were not selected for cardiovascular disease and 26 healthy female control subjects. Ascending and descending aortic diameters were measured by magnetic resonance imaging at the right pulmonary artery. TS women were on average 20 cm shorter, yet average aortic diameters were identical in the 2 groups. Ascending aortic diameters normalized to body surface area (aortic size index) were significantly greater in TS, and ≈32% of TS women had values greater than the 95th percentile of 2.0 cm/m 2 . Ascending diameter/descending diameter ratios also were significantly greater in the TS group. During ≈3 years of follow-up, aortic dissections occurred in 3 women with TS, for an annualized rate of 618 cases/100 000 woman-years. These 3 subjects had ascending aortic diameters of 3.7 to 4.8 cm and aortic size indices >2.5 cm/m 2 . Conclusions— The risk for aortic dissection is greatly increased in young women with TS. Because of their small stature, ascending aortic diameters of 2.0 cm/m 2 require close cardiovascular surveillance. Those with aortic size index ≥2.5 cm/m 2 are at highest risk for aortic dissection.Keywords
This publication has 38 references indexed in Scilit:
- Aneurysm Syndromes Caused by Mutations in the TGF-β ReceptorNew England Journal of Medicine, 2006
- Aortic Distensibility and Dimensions and the Effects of Growth Hormone Treatment in the Turner SyndromeThe American Journal of Cardiology, 2006
- Structural and functional abnormalities of large arteries in the Turner syndromeHeart, 2005
- Usefulness of Enalapril Versus Propranolol or Atenolol for Prevention of Aortic Dilation in Patients With the Marfan SyndromeThe American Journal of Cardiology, 2005
- Congenital heart disease in patients with Turner’s syndromeThe Journal of Pediatrics, 1998
- Cardiovascular Malformations and Complications in Turner SyndromePediatrics, 1998
- Aortic dilation, dissection, and rupture in patients with turner syndromeThe Journal of Pediatrics, 1986
- Pseudocoarctation of the aortic arch in a patient with turner's syndromeClinical Cardiology, 1984
- Cardiovascular malformations in Turner's and Noonan's syndrome.Heart, 1978
- Characteristic cardiovascular anomalies of XO Turner syndrome, XX and XY phenotype and XO/XX Turner mosaicThe American Journal of Cardiology, 1970