Thoracic aortopathy in Turner syndrome and the influence of bicuspid aortic valves and blood pressure: a CMR study

Abstract

BackgroundTo investigate aortic dimensions in women with Turner syndrome (TS) in relation to aortic valve morphology, blood pressure, karyotype, and clinical characteristics.Methods and resultsA cross sectional study of 102 women with TS (mean age 37.7; 18-62 years) examined by cardiovascular magnetic resonance (CMR- successful in 95), echocardiography, and 24-hour ambulatory blood pressure. Aortic diameters were measured by CMR at 8 positions along the thoracic aorta. Twenty-four healthy females were recruited as controls. In TS, aortic dilatation was present at one or more positions in 22 (23%). Aortic diameter in women with TS and bicuspid aortic valve was significantly larger than in TS with tricuspid valves in both the ascending (32.4 ± 6.7 vs. 26.0 ± 4.4 mm; p < 0.001) and descending (21.4 ± 3.5 vs. 18.8 ± 2.4 mm; p < 0.001) aorta. Aortic diameter correlated to age (R = 0.2 - 0.5; p < 0.01), blood pressure (R = 0.4; p < 0.05), a history of coarctation (R = 0.3; p = 0.01) and bicuspid aortic valve (R = 0.2-0.5; p < 0.05). Body surface area only correlated with descending aortic diameter (R = 0.23; p = 0.024).ConclusionsAortic dilatation was present in 23% of adult TS women, where aortic valve morphology, age and blood pressure were major determinants of the aortic diameter.

Cardiovascular findings and management in Turner syndrome: insights from a French cohort

“…This percentage is high, even though our observational study might have underestimated the amount of dilated aorta. The relationship between aortic dilatation and BAV was significant (P!0.001), as suggested in previous studies (13,22). All our patients with an aortic diameter O32 mm are alive and none underwent cardiac surgery between 2009 and 2011.…”

Zaburzenia w budowie aorty u pacjentek z zespołem Turnera — nowe metody diagnostyki i leczenia

“…The main risk factors for increased aortic size in TS are: hypertension, presence of BAV, aortic coarctation, and 45,X karyotype [17,34,37,43,44,46,48]. Hypertension leads to increased risk and progression of aortic dilation [23,26]: the growth rate of the aorta is about 0.07 to 0.2 cm/y but it can be accelerated by hypertension [28].…”

“…Despite a normal ascending aortic diameter in absolute terms, after indexation for body size, patients with Turner syndrome may have a dilated aorta [10,20,37,39,44].…”

“…Several studies in Denmark, France, the UK and the USA have evaluated the benefits of MRI in aortic dilation diagnosis [40][41][42][43][44][45][46]48]. Despite recent guidelines for monitoring patients with thoracic aorta lesions, the sites of measurements of aorta are not clearly defined [3,27,57].…”

“…Recent guidelines suggest that girls who have only had echocardiography should undergo MRI when old enough to cooperate with personnel during the procedure and then every 5-10 years as adults [3]. Transoesophageal ECHO is too invasive for a screening examination [40], though transthoracic echocardiography visualisation is useful for the diagnosis and follow-up of some segments of the aorta [39,44]. The value of echocardiography as a screening tool in the TS population may be suboptimal secondary to widespread abnormal chest dimensions, rendering achievement of appropriate echocardiography acoustic windows difficult [37,44].…”