Startseite Assessment of aortic morphology and compliance in children and adolescents with Ullrich-Turner syndrome (UTS) using magnetic resonance imaging (MRI)
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Assessment of aortic morphology and compliance in children and adolescents with Ullrich-Turner syndrome (UTS) using magnetic resonance imaging (MRI)

  • Carolin Kneppo , Joachim G. Eichhorn , Sebastian Ley , Jens P. Schenk , Matthias Gorenflo und Markus Bettendorf EMAIL logo
Veröffentlicht/Copyright: 22. Mai 2014

Abstract

Aim: The aim of this study was to evaluate the morphology and elastic properties of the aorta in children and adolescents with Ullrich-Turner syndrome (UTS) treated with growth hormone, by using magnetic resonance imaging (MRI).

Methods: Thirty-seven conscious UTS patients were examined using a 1.5-T whole-body MRI. Contrast-free three-dimensional (3D)-MR angiographies were performed, including 2D cine MRI, to calculate the aortic compliance (C) and cine of the aortic valve.

Results: Changes of aortic morphology were evident in 40% of the patients, whereas six had more than one alteration. A bicuspid aortic valve was identified in three patients that were missed by previous echocardiography. The aortic compliances in UTS patients were similar to those in healthy persons.

Conclusion: This study shows that aortic morphology and compliance can be assessed by MRI without using contrast agents and without sedation in children and adolescents with UTS.


Corresponding author: Markus Bettendorf, Professor MD, Division of Pediatric Endocrinology and Diabetes, University Children’s Hospital, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany, Phone: 49-6221-564002, Fax: 49-6221-564203, E-mail:
aC.K. and J.G.E. contributed equally to the manuscript. It was presented in part at the 90th annual meeting of the Endocrine Society 2008 San Francisco, USA (poster prize award), and at the 54th annual meeting of the German Society of Endocrinology 2011 in Hamburg, Germany (poster prize award).bReprint requests should be addressed to Prof. Dr. Markus Bettendorf.

References

1. Bondy CA. Turner syndrome 2008. Horm Res 2009;71:52–6.10.1159/000178039Suche in Google Scholar

2. Sachdev V, Matura LA, Sidenko S, Ho VB, Arai AE, et al. Aortic valve disease in Turner syndrome. J Am Coll Cardiol 2008;51:1904–9.10.1016/j.jacc.2008.02.035Suche in Google Scholar

3. Mortensen KH, Hjerrild BE, Stochholm K, Andersen NH, Sorensen KE, et al. Dilation of the ascending aorta in Turner syndrome – a prospective cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 2011;13:1–9.10.1186/1532-429X-13-24Suche in Google Scholar

4. Movahed MR, Hepner AD, Ahmadi-Kashani M. Echocardiographic prevalence of bicuspid aortic valve in the population. Heart Lung Circ 2006;15:297–9.10.1016/j.hlc.2006.06.001Suche in Google Scholar

5. Hoffmann JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890–900.10.1016/S0735-1097(02)01886-7Suche in Google Scholar

6. Dawson-Falk KL, Wright AM, Bakker B, Pitlick PT, Wilson DM, et al. Cardiovascular evaluation in Turner syndrome: utility of MR imaging. Australas Radiol 1992;36:204–9.10.1111/j.1440-1673.1992.tb03152.xSuche in Google Scholar PubMed

7. Bondy C. Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab 2007;92:10–25.10.1210/jc.2006-1374Suche in Google Scholar PubMed

8. Schoemaker MJ, Swerdlow AJ, Higgins CD, Wright AF, Jacobs PA. Mortality in women with Turner syndrome in Great Britain: a national cohort study. J Clin Endocrinol Metab 2008;93:4735–42.10.1210/jc.2008-1049Suche in Google Scholar PubMed

9. Bondy CA. Aortic dissection in Turner syndrome. Curr Opin Cardiol 2008;23:519–26.10.1097/HCO.0b013e3283129b89Suche in Google Scholar PubMed PubMed Central

10. van den Berg J, Bannink EM, Wielopolski PA, Pattynama PM, Muinck Keizer-Schrama SM, et al. Aortic distensibility and dimensions and the effects of growth hormone treatment in the Turner syndrome. Am J Cardiol 2006;97:1644–9.10.1016/j.amjcard.2005.12.058Suche in Google Scholar PubMed

11. Al-Mallah MH, Nasir K, Katz R, Lima JA, Bluemke DA, et al. Relation of thoracic aortic distensibility to left ventricular area (from the Multi-ethnic Study of Atherosclerosis [MESA]). Am J Cardiol 2014;113:178–82.10.1016/j.amjcard.2013.09.039Suche in Google Scholar PubMed PubMed Central

12. Metafratzi ZM, Efremidis SC, Skopelitou AS, Roos A. The clinical significance of aortic compliance and its assessment with magnetic resonance imaging. J Cardiovasc Magn Reson 2002;4:481–91.10.1081/JCMR-120016386Suche in Google Scholar

13. Mazzanti L, Lovato L, Prandstraller D, Scarano E, Tamburrino F, et al. Turner syndrome strategies to improve care outcomes – cardiac evaluation using new imaging techniques. Pediatr Endocrinol Rev 2012;9:701–9.Suche in Google Scholar

14. Mortensen KH, Hjerrild BE, Andersen NH, Sorensen KE, Horlyck A, et al. Abnormalities of the major intrathoracic arteries in Turner syndrome as revealed by magnetic resonance imaging. Cardiol Young 2010;20:191–200.10.1017/S1047951110000041Suche in Google Scholar PubMed

15. Hjerrild BE, Mortensen KH, Sorensen KE, Pedersen EM, Andersen NH, et al. Thoracic aortopathy in Turner syndrome and the influence of biscupid aortic valves and blood pressure: a CMR study. J Cardiovasc Magn Reson 2010;12:1–10.10.1186/1532-429X-12-12Suche in Google Scholar PubMed PubMed Central

16. Eichhorn JG, Krissak R, Rüdiger HJ, Ley S, Arnold R, et al. Compliance of the normal-sized aorta in adolescents with Marfan syndrome: comparison of MR measurements of aortic distensibility and pulse wave velocity. Rofo 2007;179:841–6.10.1055/s-2007-963192Suche in Google Scholar PubMed

17. Ley-Zaporozhan J, Kreitner KF, Unterhinninghofen R, Helm E, Puderbach M, et al. Assessment of thoracic aortic dimensions in an experimental setting: comparison of different unenhanced magnetic resonance angiography techniques with electrocardiogram-gated computed tomography angiography for possible application in the pediatric population. Investig Radiol 2008;43:179–86.10.1097/RLI.0b013e31815f8870Suche in Google Scholar PubMed

18. Cleeman L, Mortensen KH, Holm K, Smedegaard H, Skouby SO, et al. Aortic dimensions in girls and young women with Turner syndrome: a magnetic resonance imaging study. Pediatr Cardiol 2010;31:497–504.10.1007/s00246-009-9626-8Suche in Google Scholar PubMed

19. Ho VB, Bakalov VK, Cooley M, Van PL, Hood MN, et al. Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. Circulation 2004;110:1694–700.10.1161/01.CIR.0000142290.35842.B0Suche in Google Scholar PubMed

20. Sorensen TS, Koerperich H, Greil GF, Eichhorn J, Barth P, et al. Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease – a validation study. Circulation 2004;110:163–9.10.1161/01.CIR.0000134282.35183.ADSuche in Google Scholar PubMed

21. Boese JM, Bock M, Schoenberg SO, Schad L. Estimation of aortic compliance using magnetic resonance pulse wave velocity measurement. Phys Med Biol 2000;45:1703–13.10.1088/0031-9155/45/6/320Suche in Google Scholar PubMed

22. Sahn DJ, DeMarisa A, Kisslo J, Weyman A. Recommendations regarding quantitation in m-mode echocardiography: results of a survey of echocardiographic measurements. Circulation 1978;58:1072–83.10.1161/01.CIR.58.6.1072Suche in Google Scholar

23. Wuhl E, Witte K, Soergel M, Mehls O, Schaefer F. Distribution of 24-h ambulatory blood pressure in children: normalized reference values and role of body dimensions. J Hypertens 2002;20:1995–2007.10.1097/00004872-200210000-00019Suche in Google Scholar PubMed

24. Castro AV, Okoshi K, Ribeiro SM, Ribeiro SM, Barbosa MF, et al. Cardiovascular assessment of patients with Ullrich-Turner’s syndrome on Doppler echocardiography and magnetic resonance imaging. Ar Qbras Cardiol 2002;78:51–8.10.1590/S0066-782X2002000100005Suche in Google Scholar PubMed

25. Ostberg JE, Brookes JA, McCarthy C, Halcox J, Conway GS. A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome. J Clin Endocrinol Metab 2004;89:5966–71.10.1210/jc.2004-1090Suche in Google Scholar PubMed

26. Matura LA, Ho VB, Rosing DR, Bondy CA. Aortic dilatation and dissection in Turner syndrome. Circulation 2007;116: 1663–70.10.1161/CIRCULATIONAHA.106.685487Suche in Google Scholar PubMed

27. Pinsker JE. Turner syndrome: updating the paradigm of clinical care. J Clin Endocrinol Metab 2012;97:E994–1003.10.1210/jc.2012-1245Suche in Google Scholar PubMed

28. Aronberg DJ, Glazer HS, Madsen K, Sagel SS. Normal thoracic aortic diameters by computed tomography. J Comput Assist Tomogr 1984;8:247–50.Suche in Google Scholar

29. Carlson M, Airhart N, Lopez L, Silberbach M. Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome. Circulation 2012;126:2220–6.10.1161/CIRCULATIONAHA.111.088633Suche in Google Scholar PubMed

30. Davenport ML. Approach to the patient with Turner syndrome. J Clin Endocrinol Metab 2010;95:1487–95.10.1210/jc.2009-0926Suche in Google Scholar PubMed

31. Potthast S, Mitsumori L, Stanescu LA, Richardson ML, Branch K, et al. Measuring aortic diameter with different MR techniques: comparison of three-dimensional (3D) navigated steady-state free-precession (SSFP), 3D contrast-enhanced magnetic resonance angiography (CE-MRA), 2D T2 black blood, and 2D cine SSFP. J Magn Reson Imaging 2010;31:177–84.10.1002/jmri.22016Suche in Google Scholar PubMed

32. Barker AJ, Staehle F, Bock J, Jung BA, Markl M. Analysis of complex cardiovascular flow with three-component acceleration-encoded MRI. Magn Reson Med 2012;67:50–61.10.1002/mrm.22974Suche in Google Scholar PubMed

33. Gravholt CH, Landin-Wilhelmsen K, Stochholm K, Hjerrild BE, Ledet T, et al. Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young 2006;16:430–6.10.1017/S1047951106000928Suche in Google Scholar

34. Carlson M, Silberbach M. Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature. J Med Genet 2007;44:745–9.10.1136/jmg.2007.052019Suche in Google Scholar

35. Lin AE, Lippe B, Rosenfeld RG. Further delineation of aortic dilation, dissection, and rupture in patients with Tuner syndrome. Pediatrics 1998;102:E12.10.1542/peds.102.1.e12Suche in Google Scholar

36. Mazzanti L, Cacciari E. Congenital heart disease in patients with Turner’s syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediatr 1998;133:688–92.10.1016/S0022-3476(98)70119-2Suche in Google Scholar

37. Elsheikh M, Casadei B, Conway GS, Wass JA. Hypertension is a major risk factor for aortic root dilatation in women with Turner’s syndrome. Clin Endocrinol (Oxf) 2001;54:69–73.10.1046/j.1365-2265.2001.01154.xSuche in Google Scholar PubMed

38. Dulac Y, Pienkowski C, Abadir S, Tauber M, Acar P. Cardiovascular abnormalities in Turner’s syndrome: what prevention? Arch Cardiovasc Dis 2008;101:485–90.10.1016/j.acvd.2008.05.007Suche in Google Scholar PubMed

39. Bondy CA, Van PL, Bakalov VK, Sachdev V, Malone CA, et al. Prolongation of the cardiac QTc interval in Turner syndrome. Medicine (Baltimore) 2006;85:75–81.10.1097/01.md.0000205629.16302.bcSuche in Google Scholar PubMed

40. Bondy CA, Ceniceros I, Van PL, Bakalov VK, Rosing DR. Prolonged rate-corrected QT interval and other electrocardiogram abnormalities in girls with Turner syndrome. Pediatrics 2006;118:1220–5.10.1542/peds.2006-0776Suche in Google Scholar PubMed

Received: 2013-8-12
Accepted: 2014-4-17
Published Online: 2014-5-22
Published in Print: 2014-9-20

©2014 by De Gruyter

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