Abstract 10686: Aortic Dissection in Turner Syndrome
Objective and Background: Girls and women with Turner syndrome are at risk for aortic dissection and rupture. However the size of the aorta and the clinical characteristics among those with Turner syndrome that have had aortic dissection has received little attention.
Methods and Results: We obtained medical records from twenty Turner syndrome individuals who had aortic dissection and voluntarily participated in the International Turner Syndrome Aortic Dissection (ITSAD) Registry. Clinical characteristics and echocardiographic information were compared to unaffected TS individuals who had echo studies performed at the annual meetings of the Turner Syndrome Society of the United States (n= 320). The mean age of those with aortic dissection was 31.5 ± 9.1 yrs. 19/20 (95%) had an associated cardiac malformation. One individual had neither hypertension nor cardiac malformation. Pregnancy history was documented in 3/20 (15%). More than half (11/20) came to medical attention > 24 hours after the onset of symptoms. For those with Stanford type-A dissections the ascending aortic size index (ASI-AAO) was 2.7 ± 0.6 cm/m2 (n=9) compared to 1.8 ± 0.2 (p 2.5cm/m2 had a 95% sensitivity and a 99% specificity for predicting aortic dissection.
Conclusion: Aortic dissection in Turner syndrome occurs in young individuals and at smaller aortic diameters than the general population and those with genetically triggered aortopathy. Aortic dissection can occur in Turner syndrome without cardiac malformations or hypertension. Turner syndrome individuals who are over 18y and who have an ASI-AAO above 2.5 cm/m2 should be considered for aortic surgery to prevent aortic dissection.
- © 2012 by American Heart Association, Inc.