Abstract
To investigate intrinsic vascular abnormalities of the ascending aorta before adulthood in Turner syndrome patients (TS), we compared 25 adolescent TS patients (mean age 14.6 ± 3.4 years) and 16 healthy controls from a university hospital. Blood pressure and other vascular indices were evaluated using echocardiography such as aortic strain, stiffness index, distensibility, and pulse wave velocity. Aortic strain (0.1237 vs. 0.1865, respectively; P = 0.003) and aortic distensibility (0.0049 vs. 0.0081, P = 0.002) were significantly lower in the TS group than in the controls. The fractional area change on velocity vector imaging was also lower in TS patients (29.05 vs. 36.19, P = 0.002). These findings were still observed after adjustment for age, body mass index, and systolic blood pressure. The aortic stiffness index was greater in the TS patients than in the control group (6.79 vs. 3.34, P = 0.02). The pulse wave velocity and ascending aorta diameter did not significantly differ between the TS and control groups. Blood pressures were significantly higher in the TS patients than in the controls (systolic, 120.4 vs. 108.4 mmHg, P = 0.001; diastolic 71.5 vs. 61.7 mmHg, P < 0.001). Although the dimensional changes in the aorta were not clearly observed in adolescents with TS, the elastic properties of the aorta were significantly decreased in TS patients as compared to control subjects.
Similar content being viewed by others
References
Schoemaker MJ, Swerdlow AJ, Higgins CD, Wright AF, Jacobs PA, United Kingdom Clinical Cytogenetics Group (2008) Mortality in women with turner syndrome in Great Britain: a national cohort study. J Clin Endocrinol Metab 93(12):4735–4742
Carlson M, Silberbach M (2007) Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature. J Med Genet 44(12):745–749
Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M, Cooper JV, Januzzi JL, Ince H, Sechtem U, Bossone E, Fang J, Smith DE, Isselbacher EM, Pape LA, Eagle KA (2004) Gender-related differences in acute aortic dissection. Circulation 109(24):3014–3021
Elsheikh M, Casadei B, Conway GS, Wass JA (2001) Hypertension is a major risk factor for aortic root dilatation in women with Turner’s syndrome. Clin Endocrinol (Oxf) 54(1):69–73
Turtle EJ, Sule AA, Webb DJ, Bath LE (2015) Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations. Arch Dis Child 100(7):662–666
Lopez L, Arheart KL, Colan SD, Stein NS, Lopez-Mitnik G, Lin AE, Reller MD, Ventura R, Silberbach M (2008) Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics 121(6):e1622–e1627
Sybert VP, McCauley E (2004) Turner’s syndrome. N Engl J Med 351(12):1227–1238
National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114(2 Suppl 4th Report):555–576
Moon JS, Lee SY, Nam CM, Choi JM, Choe BK, Seo JW, Oh KW, Jang MJ, Hwang SS, Yoo MH, Kim YT, Lee CG (2008) 2007 Korean National Growth Charts: review of developmental process and an outlook. Korean J Pediatr 51(1):1–25
Warren AE, Boyd ML, O’Connell C, Dodds L (2006) Dilatation of the ascending aorta in paediatric patients with bicuspid aortic valve: frequency, rate of progression and risk factors. Heart 92(10):1496–1500
Kim KH, Park JC, Yoon HJ, Yoon NS, Hong YJ, Park HW, Kim JH, Ahn Y, Jeong MH, Cho JG, Kang JC (2009) Usefulness of aortic strain analysis by velocity vector imaging as a new echocardiographic measure of arterial stiffness. J Am Soc Echocardiogr 22(12):1382–1388
De Groote K, Devos D, Van Herck K, De Wolf D, Van der Straaten S, Rietzschel E, Raes A, Vandekerckhove K, Panzer J, De Wilde H, De Schepper J (2016) Increased aortic stiffness in prepubertal girls with Turner syndrome. J Cardiol. doi:10.1016/j.jjcc.2016.03.006
Vlachopoulos C, Aznaouridis K, Stefanadis C (2010) Prediction of cardiovascular events and all-cause mortality with arterial stiffness: a systematic review and meta-analysis. J Am Coll Cardiol 55(13):1318–1327
Tomlinson LA (2012) Methods for assessing arterial stiffness: technical considerations. Curr Opin Nephrol Hypertens 21(6):655–660
Baguet JP, Douchin S, Pierre H, Rossignol AM, Bost M, Mallion JM (2005) Structural and functional abnormalities of large arteries in the Turner syndrome. Heart 91(11):1442–1446
Lawson SA, Urbina EM, Gutmark-Little I, Khoury PR, Gao Z, Backeljauw PF (2014) Vasculopathy in the young Turner syndrome population. J Clin Endocrinol Metab 99(10):E2039–E2045
Redheuil A, Yu WC, Wu CO, Mousseaux E, de Cesare A, Yan R, Kachenoura N, Bluemke D, Lima JA (2010) Reduced ascending aortic strain and distensibility: earliest manifestations of vascular aging in humans. Hypertension 55(2):319–326
Kim SA, Lee KH, Won HY, Park S, Chung JH, Jang Y, Ha JW (2013) Quantitative assessment of aortic elasticity with aging using velocity-vector imaging and its histologic correlation. Arterioscler Thromb Vasc Biol 33(6):1306–1312
Kim HL, Im MS, Seo JB, Chung WY, Kim SH, Kim MA, Zo JH (2013) The association between arterial stiffness and left ventricular filling pressure in an apparently healthy Korean population. Cardiovascular Ultrasound 11(1):2
Akyurek N, Atabek ME, Eklioglu BS, Alp H (2014) Ambulatory blood pressure and subclinical cardiovascular disease in children with turner syndrome. Pediatr Cardiol 35(1):57–62
Ucar A, Oz F, Bas F, Oflaz H, Nisli K, Tugrul M, Yetim A, Darendeliler F, Saka N, Poyrazoglu S, Bundak R (2015) Increased arterial stiffness in young normotensive patients with Turner syndrome: associations with vascular biomarkers. Clin Endocrinol (Oxf) 82(5):719–727
Acknowledgments
This study was supported by a grant from the Korean Pediatric Heart Society.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
There are no conflicts of interest to declare.
Rights and permissions
About this article
Cite this article
An, H.S., Baek, J.S., Kim, G.B. et al. Impaired Vascular Function of the Aorta in Adolescents with Turner Syndrome. Pediatr Cardiol 38, 20–26 (2017). https://doi.org/10.1007/s00246-016-1478-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-016-1478-4