Aortic dilation and growth in women with Turner syndrome

Abstract

ObjectiveWomen with Turner syndrome (TS) are at increased risk of aortic dissection, which is a life-threatening event associated with aortic dilation. Knowledge on the development of aortic dilation over time remains limited. This study aims to describe the prevalence of aortic dilation, to find associated factors and to study aortic growth in women with TS.MethodsIn this prospective multicentre cohort study, consecutive adult women with genetically proven TS included between 2014 and 2016 underwent ECG-triggered multiphase CT angiography at baseline and after 3 years. Aortic diameters were measured at seven levels of the thoracic aorta using double oblique reconstruction and indexed for body surface area. Ascending aortic dilation was defined as an aortic size index >20 mm/m2. Aorta-related and cardiovascular events were collected. Statistical analysis included linear and logistic regression and linear mixed effects models.ResultsThe cohort consisted of 89 women with a median age of 34 years (IQR: 24–44). Ascending aortic dilation was found in 38.2% at baseline. At baseline, age (OR: 1.08 (95% CI 1.03 to 1.13), p<0.001), presence of bicuspid aortic valve (BAV) (OR: 7.09 (95% CI 2.22 to 25.9), p=0.002) and systolic blood pressure (OR: 1.06 (95% CI 1.02 to 1.11), p=0.004) were independently associated with ascending aortic dilation. During a median follow-up of 3.0 (2.4–3.6) years (n=77), significant aortic growth was found only at the sinotubular junction (0.20±1.92 mm, p=0.021). No aortic dissection occurred, one patient underwent aortic surgery and one woman died.ConclusionsIn women with TS, ascending aortic dilation is common and associated with age, BAV and systolic blood pressure. Aortic diameters were stable during a 3-year follow-up, apart from a significant yet not clinically relevant increase at the sinotubular junction, which may suggest a more benign course of progression than previously reported.

Turner syndrome: fertility counselling in childhood and through the reproductive lifespan

“…Overall mortality rates are increased approximately three-fold in women with Turner syndrome compared with the general population. There is particularly high risk for adverse cardiovascular outcomes [15,16,17 ▪ ,18]. Diagnosis is often delayed until late childhood and adolescence, which limits opportunities to screen and manage Turner syndrome comorbidities [19].…”

Assessment of aortic dilatation in Chinese children and adolescents with Turner syndrome: a single center experience

“…The ascending aorta is the most commonly affected site in TS patients compared to the sinus of Valsalva or the sino-tubular junction [ 5 ]. Various indicators are employed for evaluating AD, such as the aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and the Turner syndrome-specific Z-score (TSZ-score) [ 5 , 9 ], each with its own advantages and disadvantages. Cardiovascular imaging provides relatively clear criteria for diagnosing AD in adults: an ascending aorta ASI ≥ 2.0 cm/m 2 , A/D ratio ≥ 1.5, or ascending aorta TSZ-score ≥ 2 is defined as dilatation in adults [ 9 , 10 ].…”

“…Various indicators are employed for evaluating AD, such as the aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and the Turner syndrome-specific Z-score (TSZ-score) [ 5 , 9 ], each with its own advantages and disadvantages. Cardiovascular imaging provides relatively clear criteria for diagnosing AD in adults: an ascending aorta ASI ≥ 2.0 cm/m 2 , A/D ratio ≥ 1.5, or ascending aorta TSZ-score ≥ 2 is defined as dilatation in adults [ 9 , 10 ]. However, these standards remain controversial in pediatric clinical practice, especially regarding their applicability in Chinese children, which has not yet been verified.…”

Heartbeat: hospitalisation for COVID-19 is associated with an increased risk of subsequent adverse cardiovascular events

“…Women with Turner syndrome (TS) are at increased risk for aortic dilation and dissection, often in association with a bicuspid aortic valve. In a prospective multicentre cohort study3 of 89 women with TS, 38% had aortic dilation at baseline, with associated clinical risk factors of age (OR: 1.08 (95% CI 1.03 to 1.13), p<0.001), bicuspid aortic valve (BAV) (OR: 7.09 (95% CI 2.22 to 25.9), p=0.002) and systolic blood pressure (OR: 1.06 (95% CI 1.02 to 1.11), p=0.004). Progressive aortic dilation over a mean of 3 years follow-up was minimal with an increase of only 0.20±1.92 mm, at the sinotubular junction and there were no cases of aortic dissection (figure 2).…”

“…Galian-Gay and Rodriquez-Palomares4 comment that ‘although TS has been considered a high-risk disease for many years due to its high rate of aortic events, data from the study of Meccanici et al 3 suggest that its course could be much more benign. However, further multicentre prospective studies involving more patients are required to confirm these results and change the current understanding of the disease.’…”