Determinants of Increased Aortic Diameters in Young Normotensive Patients With Turner Syndrome Without Structural Heart Disease

Abstract

Factors associated with aortic dilation and dissection in patients with Turner syndrome (TS) remain unclear. We assessed magnetic resonance imaging-based aortic diameters at nine predefined anatomic positions and examined associations of increased aortic diameters with B-type natriuretic peptide (BNP), A-type NP (ANP), growth hormone treatment, insulin-like growth factor 1 (IGF1), and estrogen status. Forty-seven patients with TS aged 7.3-21 years and 34 healthy peers were enrolled in this study. Aortic diameters were higher in patients with TS at three positions than in controls (p < 0.05). History of GH treatment, pubertal status, and serum estradiol levels were not associated with increased aortic diameters. Patients with TS had higher plasma BNP and ANP levels than controls. BNP and IGF1 were independently associated with the increase in aortic diameters in TS at three positions of the ascending aorta (R = 0.361-0.458, p < 0.05 for all). At two positions of the descending aorta, only BNP emerged as an independent variable (R = 0.130-0.139, p < 0.05). We conclude that young, normotensive patients with TS had greater aortic diameters at several positions than healthy controls. BNP and IGF1 were independently associated with increased aortic diameters in TS.

Clinical characteristics and rate of dilatation in Turner syndrome patients treated for aortic dilatation

“…The most common karyotype was 45,X, with other karyotypes including four patients with mosaic 45,X/46,XX, three patients with 46,XiX(q10), one patient each with mosaic 45,X/46,XY and 46,Xdel(X)(p22.1), and one karyotype was unknown. As seen in other cohorts with relatively young Turner syndrome populations (Uçar et al, 2018), treatment with growth hormone was common (78% of the patients). A similar percentage of patients (73%) were treated with estrogen.…”

Identification of Key Immune Infiltration Related Genes Involved in Aortic Dissection Using Bioinformatic Analyses and Experimental Verification

“… 33 , 34 In addition, a recent study on atherosclerosis showed that IGF1 pathway could regulate the inflammation and proliferation of vascular smooth muscle cells. 35 In AD-related disease, IGF1 has been reported to be positively correlated with an increased aortic diameters 36 in Turner syndrome, which is a common cause of AD. NOS3 is a key player in governing angiogenesis, and its aberrant expression will result in the dysfunction of vascular endothelium.…”