Aortic growth rates are not increased in Turner syndrome—a prospective CMR study

Abstract

Background Aortic disease is a key determinant of outcomes in Turner syndrome (TS). The present study characterized aortic growth rates and outcomes over nearly a decade in adult women with TS. Methods and results Prospective observational study assessing aortic diameters twice with cardiovascular magnetic resonance imaging in women with TS [N = 91; mean follow-up 8.8 ± 3.3 (range 1.6–12.6) years] and healthy age-matched female controls [N = 37; mean follow-up 6.7 ± 0.5 (range 5.9–8.1) years]. Follow-up also included aortic outcomes and mortality, antihypertensive treatment and ambulatory blood pressure. Aortic growth rates were similar or smaller in TS, but the variation was larger. The proximal aorta in TS grew by 0.20 ± 0.26 (mid-ascending) to 0.32 ± 0.36 (sinuses) mm/year. This compared to 0.26 ± 0.14 (mid-ascending) and 0.32 ± 0.17 (sinuses) mm/year in the controls. During 799 years at risk, 7 suffered an aortic outcome (1 aortic death, 2 aortic dissections, 2 aortic interventions, 2 surgical aortic listings) with further 2 aortic valve replacements. At baseline, two women were excluded. One died during subacute aortic surgery (severe dilatation) and one had a previously undetected type A dissection. The combined aortic outcome rate was 1126 per 100 000 observation years. The aortic and all-cause mortality rates were 1 per 799 years (125 deaths per 100 000 observation years) and 9 per 799 years (1126 deaths per 100 000 observation years). Aortic growth patterns were particularly perturbed in bicuspid aortic valves (BAV) and aortic coarctation (CoA). Conclusion Aortic growth rates in TS are not increased. BAVs and CoA are major factors that impact aortic growth. Aortic outcomes remain a concern.

Heart and Turner syndrome

“…Few longitudinal studies have evaluated the aortic diameter progression in large cohorts of TS patients. Mortensen et al [19] found in 91 patients with TS, that the aortic progression at the level of the Valsalva sinuses and the tubular ascending levels were 0.32-±0.3 mm/y and 0.20-±0.2 mm/y, respectively, after a mean follow-up of 8.8 years. A control population of 37 women was included in the study.…”

Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years

“…This is currently lacking in the United States (Lin et al, ), but under development as a self‐reporting patient registry (Prakash, Lugo‐Ruiz, et al, ). In addition to outcome data focusing on obstetric and fetal/neonatal morbidity, it will be important to extend ongoing population‐based studies of aortic growth (Mortensen et al, ) to assess whether pregnancy has an effect. A recent prospective follow‐up study showed that aortic growth rates in adult women with Turner syndrome overall are similar to healthy female controls.…”

“…However, women with a BAV or repaired coarctation of the aorta appeared to dilate more rapidly. Interestingly, planned aortic root or valve surgery was a considerable cause of morbidity and mortality in this cohort (Mortensen et al, ).…”

Prevalence of Bicuspid Aortic Valve in Turner Syndrome Patients Receiving Cardiac MRI and CT: A Meta-Analysis

“…According to the latest clinical practice guidelines for the care of girls and women with TS, bicuspid aortic valve (BAV) is the most common CHD in TS affecting between 14-34% of individuals [1]. Aortic dilatation and aortic dissection, which are both associated with the presence of BAV, are common in TS [5]. The management of these acquired aortic conditions is crucial for the cardiovascular outcome of TS patients [6].…”