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Published on: March 28, 2025
Aortic dilatation and dissection in Turner syndrome
Lea Ann Matura1, Vincent B Ho, Douglas R Rosing
1Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Women with Turner syndrome (TS) have an increased risk of aortic dissection. Aortic size index > 2.0 cm/m2 indicates significant aortic dilatation and requires close monitoring for dissection risk.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Endocrinology
Background:
- Turner syndrome (TS) is associated with an elevated risk of aortic dissection in young women.
- The relationship between aortic dilatation and acute aortic dissection in TS remains unclear.
- Predictors of aortic deterioration in TS patients require further investigation.
Purpose of the Study:
- To investigate whether aortic dilatation precedes aortic dissection in women with Turner syndrome.
- To identify specific aortic diameter measurements that predict impending aortic events in TS.
- To assess the utility of aortic size index in managing cardiovascular risk in TS.
Main Methods:
- Retrospective analysis of 166 adult women with TS and 26 healthy female controls.
- Ascending and descending aortic diameters measured via MRI, normalized to body surface area (aortic size index).
- Follow-up for approximately 3 years to record aortic dissection events.
Main Results:
- Women with TS had similar absolute aortic diameters but significantly greater aortic size indices compared to controls.
- 32% of TS women exhibited aortic size index values above the 95th percentile (> 2.0 cm/m2).
- Three TS women experienced aortic dissections, all with ascending aortic diameters > 3.7 cm and aortic size indices > 2.5 cm/m2.
Conclusions:
- Aortic dissection risk is substantially increased in young women with TS.
- Aortic size index is a preferred measure for detecting dilatation in TS due to their shorter stature.
- Aortic size index > 2.0 cm/m2 warrants close cardiovascular surveillance; > 2.5 cm/m2 indicates the highest risk for dissection.
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