Author + information
- Colleen Pater,
- Iris Gutmark-Little,
- Justin Tretter,
- Lisa Martin,
- Philippe Backeljauw and
- Nicole Brown
Turner Syndrome (TS) is associated with an increased risk of aortic aneurysms and dissection (at median age 29-35 years). Additional risk factors include bicuspid aortic valve (BAV), coarctation and hypertension. Experts suggest optimizing medical therapy for rapid growth, but the rate of growth and whether treatment is effective in slowing it is unknown. The aim of this study was to describe pertinent clinical information for TS patients with aortopathy, the rate of dilation and to determine if therapy affects the rate in TS patients.
We conducted a retrospective chart review of TS patients with a dilated aortic root or ascending aorta by current definitions: 1) Turner-specific Z-score ≥ 3 if < 15 yo, 2) aortic size index ≥ 2.0 cm/m2 if ≥ 15 yo, or 3) absolute aortic root diameter > 3.3 cm or ascending aortic diameter > 3.1 cm. Demographic and medication data was collected. Imaging measurements were performed by a common reader. The rates of aortic dilation in echoes and MRIs were analyzed independently.
A total of 42 patients were included for analysis, with 22 patients receiving treatment at any point during the study period. Average length of follow-up was 5.4 years, from dilation diagnosis to last imaging. Most patients had 45XO karyotype, were white, non-Hispanic and were treated with both growth hormone and estrogen. Except for hypertension, there were no statistically significant differences in risk factors for dilation among the treated and untreated groups. BAVs were very common in this cohort (73% of treated, 65% of untreated). If treated, most patients received atenolol or losartan. Rates of dilation were slow, with means less than 0.2cm/year in root and ascending aorta measurements by echo and MRI. There were no statistically significant differences in the rates of dilation between the treatment and no treatment groups.
TS patients with aortic root dilation and risk factors for dissection, particularly BAV and hypertension, are often treated with medications. The rate of aortic dilation is slow, and this small cohort suggests that the rate of change is not affected by medical therapy. A prospective study is needed to assess the utility of this common practice.
Poster Hall, Hall F
Saturday, March 16, 2019, 10:00 a.m.-10:45 a.m.
Session Title: Congenital Heart Disease: Therapy 1
Abstract Category: 11. Congenital Heart Disease: Therapy
Presentation Number: 1141-496
- 2019 American College of Cardiology Foundation