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Clinical Characteristics and Rate of Dilation in Turner Syndrome Patients Treated for Aortic Dilatation

Pater, Colleen
http://orcid.org/0000-0002-8788-8991
http://rave.ohiolink.edu/etdc/view?acc_num=ucin1563872367349462

Abstract Details

2019, MS, University of Cincinnati, Medicine: Clinical and Translational Research.
Background: Turner Syndrome (TS) is associated with an increased risk of aortic aneurysms and dissection due to aortic dilatation. Additional risk factors include bicuspid aortic valve (BAV), coarctation, and hypertension. Following diagnosis of dilatation, medical therapy is currently recommended, though whether treatment slows dilation is unknown. Our aim was to describe a historical cohort of TS patients with aortic dilatation, the rate of dilation following diagnosis, and to assess if therapy affects dilation. Methods: We conducted a retrospective chart and imaging review of TS patients with a dilated aortic root or ascending aorta by current recommendations. Results: A total of 40 patients were included, with 22 patients receiving treatment during the study period. Most patients had 45,X karyotype, were white, non-Hispanic, and were treated with both growth hormone and estrogen. Except for hypertension, there were no differences in risk factors among the treated and untreated groups. BAVs were very common in both (72.7% in treated group, 61.1% in untreated group; p =0.04). Those in the treatment group had significantly more dilated ascending aortas, by absolute measurements (mean of 2.98cm in treated group, 2.48cm in untreated group; p < 0.001) and aortic size index (mean of 2.34 in treated group, 2.01 in untreated group; p = 0.002). In order to adjust for dilation associated with known risk factors such as BAV, hypertension, and coarctation, a predictive model was created to compare expected dilation with actual dilation, and to assess whether medications were effective at slowing the rate of dilation. In this model, there was, there was minimal change in aortic measures over time since baseline (aortic root effect estimate -0.005, p = 0.46; ascending aorta effect estimate +0.006, p = 0.37). Notably, there was also no significant change in expected growth over time following medication initiation (aortic root effect estimate -0.0001, p = 0.99; ascending aorta effect estimate -0.004, p = 0.54). Conclusions: Patients with TS and aortic dilation were more likely to be treated with medications if they had hypertension at the time of diagnosis, and if they met diagnostic criteria by multiple indices. Expert opinion currently recommends treatment of aortic dilation in TS patients. However, in this small cohort, the rate of aortic dilation does not seem to be significantly affected by medications.
Patrick Ryan, Ph.D. (Committee Chair)
Nicole Brown, M.D. (Committee Member)
Lisa Martin, Ph.D. (Committee Member)
25 p.
Surgery
Turner syndrome; aortic dilatation

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Citations

  • Pater, C. (2019). Clinical Characteristics and Rate of Dilation in Turner Syndrome Patients Treated for Aortic Dilatation [Master's thesis, University of Cincinnati]. OhioLINK Electronic Theses and Dissertations Center. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1563872367349462

    APA Style (7th edition)

  • Pater, Colleen. Clinical Characteristics and Rate of Dilation in Turner Syndrome Patients Treated for Aortic Dilatation. 2019. University of Cincinnati, Master's thesis. OhioLINK Electronic Theses and Dissertations Center, http://rave.ohiolink.edu/etdc/view?acc_num=ucin1563872367349462.

    MLA Style (8th edition)

  • Pater, Colleen. "Clinical Characteristics and Rate of Dilation in Turner Syndrome Patients Treated for Aortic Dilatation." Master's thesis, University of Cincinnati, 2019. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1563872367349462

    Chicago Manual of Style (17th edition)

ucin1563872367349462
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This open access ETD is published by University of Cincinnati and OhioLINK.