TURNER SYNDROME – AN UNUSUAL CAUSE FOR AORTIC DISSECTION
DOI:
https://doi.org/10.48750/acv.306Keywords:
Turner Syndrome, Aortic Dissection, X Chromosome, Bicuspid Aortic Valve, CoarctationAbstract
Turner syndrome (TS) is a disorder of female development with cardinal features of short stature and congenital cardiovascular defects. Congenital or acquired cardiological problems occur commonly in TS, being potentially progressive and responsible for severe complications, such as aortic dissection in young women. Accordingly, we describe a case of type A aortic dissection occurring in a woman with TS, highlighting the need to prioritize investigation in those patients to avoid a catastrophic aortic scenario.
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References
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2. Ostberg JE, Conway GS. Adulthood in women with Turner’s syndrome. Horm Res 2003;59:211—21.
3. Bondy CA, Turner’s Syndrome Study Group. Care of girls and women with Turner’s syndrome: a guideline of the Turner’s syndrome study group. J Clin Endocrinol Metab 2007;92: 10—25.
4. Giordano R, Forno D, Lanfranco F et al. Metabolic and cardiovascular outcomes in a group of adult patients with Turner’s syndrome under hormonal replacement therapy. Eur J Endocrinol 2011; 164: 819–886.
5. Turtle EJ, Sule AA, Bath LE, et al. Assessing and addressing cardiovascular risk in adults with Turner syndrome. Clin Endocrinol. 2013;78:639-45.
6. Gravholt CH, Landin-Wilhelmsen K, Stochholm K, et al. Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young. 2006;16:430-6.
7. Kölbel T, Carpenter S, Lohrenz C et al. Addressing persistent false lumen flow in chronic aortic dissection: the knickerbocker technique. J Endovasc Ther. 2014. 21(1):117-22.
8. Bondy C. Pregnancy and cardiovascular risk for women with Turner syndrome. Women Health. 2015;4:1-9.
9. Stochholm K, Juul S, Juel K, Naeraa RW, Gravholt CH. Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 2006;91:3897–902.
10. 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.
11. Hjerrild BE, Mortensen KH, Sorensen KE, Pedersen EM, Andersen NH, 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.
12. Kim HK, Gottliebson W, Hor K, Backeljauw P, Gutmark-Little I et al. Cardiovascular anomalies in Turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population. AJR Am J Roentgenol 2011;196:454–60.
13. Bondy CA. Aortic dissection in Turner syndrome. Curr Opin Card 2008;23:519–26.
14. Marin A, Weir-McCall JR, Webb DJ, et al. Imaging of cardiovascular risk in patients with Turner’s syndrome. Clin Radiol. 2015;70:803-14.
15. Mokashi SA, Svensson LG. Guidelines for the management of thoracic aortic disease in 2017. Gen Thorac Cardiovasc Surg. 2017.
16. Wong SC, Cheung M, Zacharin M. Aortic dilatation and dissection in Turner syndrome: What we know, what we are unclear about and what we should do in clinical practice? International Journal of Adolescent Medicine and Health [Internet]. 2014 Jan 31.
17. 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.
18. Hagman A, Kallen K, Bryman I, Landin-Wilhelmsen K, Barrenas ML, et al. Morbidity and mortality after childbirth in women with Turner karyotype. Hum Reprod 2013;28:1961–73.
19. Karnis MF, Zimon AE, Lalwani SI, Timmreck LS, Klipstein S, et al. Risk of death in pregnancy achieved through oocyte donation in patients with Turner syndrome: a national survey. Fertil Steril 2003;80:498–501.
20. Kriksciuniene R, Ostrauskas R, Zilaitiene B. Aortopathies in Turner syndrome — new strategies for evaluation and treatment. Endokrynologia Polska. 2015 Mar 2;66(1):58–65.
21. Klein LW, Levin JL, Weintraub WS, Agarwal JB, Helfant RH. Pseudocoarctation of the aortic arch in a patient with Turner’s syndrome. Clin Cardiol. 1984;7:621–623.
22. Burgess BJ, Iftikhar K. Aortic dissection in a case of Turner’s syndrome. Emerg Med J. 2009;26:908.
23. Matura LA, Ho VB, Rosing DR, Bondy CA. Aortic dilatation and dissection in Turner syndrome. Circulation. 2007;116:1663–1670.
24. Donadille B, Rousseau A, Zenaty D et al. Cardiovascular findings and management in Turner syndrome: insights from a French cohort. Eur J Endocrinol 2012; 167: 517–522.
25. Evangelista A, Flachskampf FA, Erbel R et al.; European Association of Echocardiography; Document Reviewers:Pepi M, Breithardt OA, Plonska-Gosciniak E. Echocardiography in aortic diseases: EAE recommendations for clinical practice. Eur J Echocardiogr 2010; 11: 645–658.
26. Terry C, Yazar O, Heye S, Fourneau I. TEVAR for Ruptured type B Aortic Dissection in a Patient with Turner Syndrome, Acta Chirurgica Belgica. 2015. 115:4, 314-316.
2. Ostberg JE, Conway GS. Adulthood in women with Turner’s syndrome. Horm Res 2003;59:211—21.
3. Bondy CA, Turner’s Syndrome Study Group. Care of girls and women with Turner’s syndrome: a guideline of the Turner’s syndrome study group. J Clin Endocrinol Metab 2007;92: 10—25.
4. Giordano R, Forno D, Lanfranco F et al. Metabolic and cardiovascular outcomes in a group of adult patients with Turner’s syndrome under hormonal replacement therapy. Eur J Endocrinol 2011; 164: 819–886.
5. Turtle EJ, Sule AA, Bath LE, et al. Assessing and addressing cardiovascular risk in adults with Turner syndrome. Clin Endocrinol. 2013;78:639-45.
6. Gravholt CH, Landin-Wilhelmsen K, Stochholm K, et al. Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young. 2006;16:430-6.
7. Kölbel T, Carpenter S, Lohrenz C et al. Addressing persistent false lumen flow in chronic aortic dissection: the knickerbocker technique. J Endovasc Ther. 2014. 21(1):117-22.
8. Bondy C. Pregnancy and cardiovascular risk for women with Turner syndrome. Women Health. 2015;4:1-9.
9. Stochholm K, Juul S, Juel K, Naeraa RW, Gravholt CH. Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 2006;91:3897–902.
10. 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.
11. Hjerrild BE, Mortensen KH, Sorensen KE, Pedersen EM, Andersen NH, 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.
12. Kim HK, Gottliebson W, Hor K, Backeljauw P, Gutmark-Little I et al. Cardiovascular anomalies in Turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population. AJR Am J Roentgenol 2011;196:454–60.
13. Bondy CA. Aortic dissection in Turner syndrome. Curr Opin Card 2008;23:519–26.
14. Marin A, Weir-McCall JR, Webb DJ, et al. Imaging of cardiovascular risk in patients with Turner’s syndrome. Clin Radiol. 2015;70:803-14.
15. Mokashi SA, Svensson LG. Guidelines for the management of thoracic aortic disease in 2017. Gen Thorac Cardiovasc Surg. 2017.
16. Wong SC, Cheung M, Zacharin M. Aortic dilatation and dissection in Turner syndrome: What we know, what we are unclear about and what we should do in clinical practice? International Journal of Adolescent Medicine and Health [Internet]. 2014 Jan 31.
17. 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.
18. Hagman A, Kallen K, Bryman I, Landin-Wilhelmsen K, Barrenas ML, et al. Morbidity and mortality after childbirth in women with Turner karyotype. Hum Reprod 2013;28:1961–73.
19. Karnis MF, Zimon AE, Lalwani SI, Timmreck LS, Klipstein S, et al. Risk of death in pregnancy achieved through oocyte donation in patients with Turner syndrome: a national survey. Fertil Steril 2003;80:498–501.
20. Kriksciuniene R, Ostrauskas R, Zilaitiene B. Aortopathies in Turner syndrome — new strategies for evaluation and treatment. Endokrynologia Polska. 2015 Mar 2;66(1):58–65.
21. Klein LW, Levin JL, Weintraub WS, Agarwal JB, Helfant RH. Pseudocoarctation of the aortic arch in a patient with Turner’s syndrome. Clin Cardiol. 1984;7:621–623.
22. Burgess BJ, Iftikhar K. Aortic dissection in a case of Turner’s syndrome. Emerg Med J. 2009;26:908.
23. Matura LA, Ho VB, Rosing DR, Bondy CA. Aortic dilatation and dissection in Turner syndrome. Circulation. 2007;116:1663–1670.
24. Donadille B, Rousseau A, Zenaty D et al. Cardiovascular findings and management in Turner syndrome: insights from a French cohort. Eur J Endocrinol 2012; 167: 517–522.
25. Evangelista A, Flachskampf FA, Erbel R et al.; European Association of Echocardiography; Document Reviewers:Pepi M, Breithardt OA, Plonska-Gosciniak E. Echocardiography in aortic diseases: EAE recommendations for clinical practice. Eur J Echocardiogr 2010; 11: 645–658.
26. Terry C, Yazar O, Heye S, Fourneau I. TEVAR for Ruptured type B Aortic Dissection in a Patient with Turner Syndrome, Acta Chirurgica Belgica. 2015. 115:4, 314-316.
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Published
2021-03-05
How to Cite
1.
Augusto R, Campos J, Coelho A, Coelho N, Pinto E, Semião C, Ribeiro J, Brandão D, Canedo A. TURNER SYNDROME – AN UNUSUAL CAUSE FOR AORTIC DISSECTION. Angiol Cir Vasc [Internet]. 2021 Mar. 5 [cited 2024 Apr. 17];16(4):322-6. Available from: https://acvjournal.com/index.php/acv/article/view/306
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Clinical Case
