Cardiac MRI and 3D contrast-enhanced MR angiography in pediatric and young adult patients with Turner syndrome

Cardiac MRI and 3D contrast-enhanced MR angiography in pediatric and young adult patients with Turner syndrome

Background/aim: This study aimed to describe the spectrum and frequency of cardiovascular abnormalities in pediatric and young adult patients with Turner syndrome (TS) using cardiac MRI and MR angiography. Materials and methods: This prospective study consisted of 47 female patients of pediatric age and young adults with a karyotypically confirmed diagnosis of TS. All patients underwent cardiac MRI and contrast-enhanced MR angiography. A second examination after 9 26 months was performed for 28 of these patients. Results: Elongation of the transverse aortic arch (ETA) was the most frequent abnormality with a rate of 37%. The rate of partial anomalous pulmonary venous connection (PAPVC) was 21.7%, bicuspid aortic valve (BAV) was 19.6%, coarctation was 6.5%, ascending aorta dilatation was 28.3%, and descending aorta dilatation was 15.2%. The diameters of the aorta and the rate of aortic dilatation per unit of time was greater in the patients with BAV (P < 0.05). ETA was less observed in the patients who were receiving growth hormone therapy (P < 0.05). Conclusion: The most common cardiovascular abnormalities in TS patients are aortic arch anomalies such as ETA and coarctation, aortic dilatation, PAPVCs, and BAV. The presence of BAV is an important risk factor for the aortic dilatation.

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  • 1. Pinsker JE. Clinical review: Turner syndrome: updating the paradigm of clinical care. J Clin Endocrinol Metab 2012; 97: E994-E1003.
  • 2. Olivieri LJ, Baba RY, Arai AE, Bandettini WP, Rosing DR, Bakalov V, Sachdev V, Bondy CA. Spectrum of aortic valve abnormalities associated with aortic dilation across age groups in Turner syndrome. Circ Cardiovasc Imaging 2013; 6: 1018- 1023.
  • 3. Bondy CA; Turner Syndrome Study Group. Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab 2007; 92 :10- 25.
  • 4. Gutmark-Little I, Backeljauw PF. Cardiac magnetic resonance imaging in Turner syndrome. Clin Endocrinol (Oxf) 2013; 78: 646-658.
  • 5. Lanzarini L, Larizza D, Prete G, Calcaterra V, Meloni G, Sammarchi L, Klersy C. Aortic dimensions in Turner’s syndrome: two-dimensional echocardiography versus magnetic resonance imaging. J Cardiovasc Med (Hagerstown) 2007; 8: 428-437.
  • 6. Dawson-Falk KL, Wright AM, Bakker B, Pitlick PT, Wilson DM, Rosenfeld RG. Cardiovascular evaluation in Turner syndrome: utility of MR imaging. Australas Radiol 1992; 36: 204-209.
  • 7. Bondy CA. Turner syndrome 2008. Horm Res 2009; 71 (Suppl. 1): 52-56.
  • 8. Kim HK, Gottliebson W, Hor K, Backeljauw P, GutmarkLittle I, Salisbury SR, Racadio JM, Helton-Skally K, Fleck R. Cardiovascular anomalies in Turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population. AJR Am J Roentgenol 2011; 196: 454-460.
  • 9. Castro AV, Okoshi K, Ribeiro SM, Barbosa MF, Mattos PF, Pagliare L, Bueno NF, Rodrigueiro DA, Haddad AL. Cardiovascular assessment of patients with Ullrich-Turner’s syndrome on Doppler echocardiography and magnetic resonance imaging. Arq Bras Cardiol 2002; 78: 51-58.
  • 10. Cleemann L, Mortensen KH, Holm K, Smedegaard H, Skouby SO, Wieslander SB, Leffers AM, Leth-Espensen P, Pedersen EM, Gravholt CH. Aortic dimensions in girls and young women with turner syndrome: a magnetic resonance imaging study. Pediatr Cardiol 2010; 31: 497-504.
  • 11. van den Berg J, Bannink EM, Wielopolski PA, Hop WC, van Osch-Gevers L, Pattynama PM, de Muinck Keizer-Schrama SM, Helbing WA. Cardiac status after childhood growth hormone treatment of Turner syndrome. J Clin Endocrinol Metab 2008; 93: 2553-2558.
  • 12. Hom JJ, Ordovas K, Reddy GP. Velocity-encoded cine MR imaging in aortic coarctation: functional assessment of hemodynamic events. Radiographics 2008; 28: 407-416.
  • 13. Kaiser T, Kellenberger CJ, Albisetti M, Bergsträsser E, Valsangiacomo Buechel ER. Normal values for aortic diameters in children and adolescents--assessment in vivo by contrastenhanced CMR-angiography. J Cardiovasc Magn Reson 2008; 10:56.
  • 14. Ho VB, Bakalov VK, Cooley M, Van PL, Hood MN, Burklow TR, Bondy CA. Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. Circulation 2004; 110:1694-1700.
  • 15. Mosteller RD. Simplified calculation of body surface area. N Engl J Med 1987; 317: 1098.
  • 16. Gutgesell HP, Rembold CM. Growth of the human heart relative to body surface area. Am J Cardiol 1990; 65: 662-668.
  • 17. Sluysmans T, Colan SD. Structural measurements and adjustment for growth. In: Lai WW, Mertens L, Cohen MS, Geva T, editors. Echocardiography in Pediatric and Congenital Heart Disease, from Fetus to Adult. Oxford, UK: Blackwell Publishing Ltd.; 2009. pp. 51-62.
  • 18. Mortensen KH, Hjerrild BE, Andersen NH, Sørensen KE, Hørlyck A, Pedersen EM, Lundorf E, Christiansen JS, Gravholt CH. Abnormalities of the major intrathoracic arteries in Turner syndrome as revealed by magnetic resonance imaging. Cardiol Young 2010; 20: 191-200.
  • 19. 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: 5966-5971.
  • 20. Smyth PT, Edwards JE. Pseudocoarctation, kinking or buckling of the aorta. Circulation 1972; 46: 1027-1032.
  • 21. Nielsen JC, Powell AJ, Gauvreau K, Marcus EN, Prakash A, Geva T. Magnetic resonance imaging predictors of coarctation severity. Circulation 2005; 111: 622-628.
  • 22. Secchi F, Iozzelli A, Papini GD, Aliprandi A, Di Leo G, Sardanelli F. MR imaging of aortic coarctation. Radiol Med 2009; 114: 524- 537.
  • 23. Gutmark-Little I, Hor KN, Cnota J, Gottliebson WM, Backeljauw PF. Partial anomalous pulmonary venous return is common in Turner syndrome. J Pediatr Endocrinol Metab 2012; 25: 435-440.
  • 24. Bechtold SM, Dalla Pozza R, Becker A, Meidert A, Döhlemann C, Schwarz HP. Partial anomalous pulmonary vein connection: an underestimated cardiovascular defect in Ullrich-Turner syndrome. Eur J Pediatr 2004; 163: 158-162.
  • 25. Sachdev V, Matura LA, Sidenko S, Ho VB, Arai AE, Rosing DR, Bondy CA. Aortic valve disease in Turner syndrome. J Am Coll Cardiol 2008; 51: 1904-1909.
  • 26. Hahn RT, Roman MJ, Mogtader AH, Devereux RB. Association of aortic dilation with regurgitant, stenotic and functionally normal bicuspid aortic valves. J Am Coll Cardiol 1992; 19: 283- 238.
  • 27. Ferencik M, Pape LA. Changes in size of ascending aorta and aortic valve function with time in patients with congenitally bicuspid aortic valves. Am J Cardiol 2003; 92: 43-46.
  • 28. Mortensen KH, Hjerrild BE, Stochholm K, Andersen NH, Sørensen KE, Lundorf E, Hørlyck A, Pedersen EM, Christiansen JS, Gravholt CH. Dilation of the ascending aorta in Turner syndrome - a prospective cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 2011; 13: 24.
  • 29. Matura LA, Ho VB, Rosing DR, Bondy CA. Aortic dilatation and dissection in Turner syndrome. Circulation 2007; 116: 1663- 1670.
  • 30. Hjerrild BE, Mortensen KH, Sørensen KE, Pedersen EM, Andersen NH, Lundorf E, Hansen KW, Hørlyck A, Hager A, Christiansen JS et al. Thoracic aortopathy in Turner syndrome and the influence of bicuspid aortic valves and blood pressure: a CMR study. J Cardiovasc Magn Reson 2010; 12: 12.
  • 31. van den Berg J, Bannink EM, Wielopolski PA, Pattynama PM, de Muinck Keizer-Schrama SM, Helbing WA. Aortic distensibility and dimensions and the effects of growth hormone treatment in the turner syndrome. Am J Cardiol 2006; 97: 1644-1649.
  • 32. Bondy CA, Van PL, Bakalov VK, Ho VB. Growth hormone treatment and aortic dimensions in Turner syndrome. J Clin Endocrinol Metab 2006; 91: 1785-1788.
Turkish Journal of Medical Sciences-Cover
  • ISSN: 1300-0144
  • Yayın Aralığı: Yılda 6 Sayı
  • Yayıncı: TÜBİTAK