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Aortic dilatation in patients with Turner’s syndrome without structural cardiac anomaly

Published online by Cambridge University Press:  29 May 2015

Nassiba Alami Laroussi*
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
Nagib Dahdah
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
Frédéric Dallaire
Affiliation:
Department of Pediatrics, CHU Sherbrooke, Sherbrooke, Quebec, Canada
Johanne Thérien
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
Anne Fournier
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
*
Correspondence to: N. Alami Laroussi, Pediatric cardiology, CHU Sainte Justine, 3175 chemin de la Côte Sainte Catherine, Montreal, Quebec H3T 1C5, Canada. Tel: 514-345-4931; Fax: 514-345-4896; E-mail: [email protected]

Abstract

Introduction

Dilatation of the ascending aorta is described in Turner’s syndrome with variable prevalence (6.8–32%). Reported series typically include patients with associated cardiac anomalies.

Objective

To characterise the prevalence, age of onset, and the progress of dilatation of the ascending aorta in Turner’s syndrome patients free of structural cardiac anomalies. Potential risk factors such as karyotype and growth hormone therapy were analysed for correlation with aortic dilatation.

Methods

We carried out a retrospective study with data collected from medical records and echocardiography studies. Patients with Tuner’s syndrome followed-up between 1992 and 2010 with at least two echocardiography studies were eligible. Patients with previous cardiac surgery or under anti-hypertensive medication were excluded. Ascending aorta diameter measurements were adjusted for body surface area, and dilatation was defined as Z-score>2.

Results

The study population consisted of 44 patients, aged 11.9±7.4 years at the first echocardiogram and 17.9±7.3 years at the last follow-up, with a follow-up duration of 6.0±3.7 years. A total of 13 (29.5%) patients exhibited aortic dilatation during follow-up, suggesting an actuarial estimate of the freedom from aortic dilatation dropping from 86 to 70% and then to 37% at 10, 20, and 30 years of age, respectively. There was no statistically significant impact of karyotype or growth hormone therapy on aortic Z-score progression.

Conclusion

The prevalence of dilatation of the ascending aorta in Turner’s syndrome patients free of structural aortic anomalies is comparable with published data with associated lesions. Growth hormone therapy and karyotype had no significant impact; however, longitudinal follow-up is warranted.

Type
Original Articles
Copyright
© Cambridge University Press 2015 

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References

1. Gotzsche, CO, Krag-Olsen, B, Nielsen, J, Sorensen, KE, Kristensen, BO. Prevalence of cardiovascular malformations and association with karyotypes in Turner’s syndrome. Arch Dis Child 1994; 71: 433436.Google Scholar
2. Haddad, HM, Wilkins, L. Congenital anomalies associated with gonadal aplasia. Pediatrics 1959; 23: 885902.Google Scholar
3. Manzatti, L, Prandstraller, D, Tassinari, D, et al. Heart disease in Turner’s syndrome. Helv Paediatr Acta 1988; 43: 2531.Google Scholar
4. Hou, JW, Hwu, WL, Tsai, WY, Lee, JS, Wang, TR, Lue, HC. Cardiovascular disorders in Turner’s and its correlation to karyotype. J Formos Med Assoc 1993; 92: 188189.Google Scholar
5. Matura, LA, Ho, VB, Rosing, DR, Bondy, CA. Aortic dilatation and dissection in Turner syndrome. Circulation 2007; 116: 16631670.Google Scholar
6. Lin, AE, Lippe, B, Rosenfeld, RG. Futher delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome. Pediatrics 1998; 102: e12.CrossRefGoogle Scholar
7. 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: 745749.CrossRefGoogle ScholarPubMed
8. Gravholt, CH, Landing-Wilhelmsem, K, Stochholm, K. Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young 2006; 16: 430436.Google Scholar
9. Warren, AE, Boyd, ML, O’Connell, C, Dodds, L. Dilatation of the ascending aorta in paediatric patients with bicuspid aortic valve: frequency, rate of progression and risk factors. Heart 2006; 92: 14961500.CrossRefGoogle ScholarPubMed
10. Altit, G, Sarquella-Brugada, G, Dahdah, N, et al. Effect of dual-chamber pacemaker implantation on aortic dilatation in patients with congenital heart block. Am J Cardiol 2014; 114: 15731577.Google Scholar
11. Dallaire, F, Bigras, JL, Prsa, M, Dahdah, N. Bias related to body mass index in pediatric echocardiographic Z scores. Pediatr Cardiol 2015; 36: 667676.Google Scholar
12. Hjerrild, BE, Mortensen, KH, Sorensen, KE, 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.Google Scholar
13. Cleemann, L, Mortensen, KH, Holm, K, et al. Aortic dimensions in girls and young women with turner syndrome: a magnetic resonance imaging study. Pediatr Cardiol 2010; 4: 497504.CrossRefGoogle Scholar
14. Dore, A, Brochu, MC, Baril, JF, et al. Progressive dilation of the diameter of the aortic root in adults with a bicuspid aortic valve. Cardiol Young 2003; 13: 526531.CrossRefGoogle ScholarPubMed
15. Kim, M, Roman, MJ, Cavallini, MC, et al. Effect of hypertension on aortic root size and prevalence of aortic regurgitation. Hypertension 1996; 28: 4752.CrossRefGoogle ScholarPubMed
16. Lopez, L, Arheart, KL, Colan, SD, et al. Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics 2008; 121: e16221627.CrossRefGoogle ScholarPubMed
17. Sachdev, V, Matura, LA, Sidenko, S, et al. Aortic valve disease in Turner syndrome. J Am Coll Cardiol 2008; 51: 19041909.Google Scholar
18. Prandstraller, D, Mazzanti, L, Giardini, A, et al. Correlations of phenotype and genotype in relation to morphologic remodelling of the aortic root in patients with Turner’s syndrome. Cardiol Young 2009; 19: 264271.CrossRefGoogle ScholarPubMed
19. Gautier, M, Detaint, D, Fermanian, C, et al. Nomograms for aortic root diameters in children using two-dimensional echocardiography. Am J Cardiol 2010; 105: 888894.CrossRefGoogle ScholarPubMed
20. Lanzarini, L, Larizza, D, Prete, G, Calcaterra, V, Klersy, C. Prospective evaluation of aortic dimensions in Turner syndrome: a 2-dimensional echocardiographic study. J Am Soc Echocardiogr 2007; 3: 307313.Google Scholar
21. Mortensen, KH, Hjerrild, BE, Stochholm, K, et al. Dilation of the ascending aorta in Turner syndrome – a prospective cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 2011; 13: 24.Google Scholar
22. Irving, RJ, Carson, MN, Webb, DJ, Walker, BR. Peripheral vascular structure and function in men with contrasting GH levels. J Clin Endocrinol Metab 2002; 87: 33093314.Google Scholar
23. Smith, JC, Evans, LM, Wilkinson, I, et al. Effects of GH replacement on endothelial function and large artery stiffness in GH-deficient adults: a randomized, double blinded, placebo controlled study. Clin Endocrinol (Oxf) 2002; 56: 493501.CrossRefGoogle Scholar
24. Bruel, A, Oxlund, H. Growth hormone influences the content and composition of collagen in the aorta from old rats. Mech Ageing Dev 2002; 123: 627635.CrossRefGoogle ScholarPubMed
25. Bondy, CA, Van, PL, Bakalov, VK, Ho, VB. Growth hormone treatment and aortic dimension in Turner syndrome. J Clin Endocrinol Metab 2006; 91: 17851788.CrossRefGoogle ScholarPubMed
26. Matura, LA, Sachdev, V, Bakalov, VK, Rosing, DR, Bondy, CA. Growth hormone treatment and left ventricular dimensions in Turner syndrome. J Pediatr 2007; 150: 587591.Google Scholar
27. Van den Berg, J, Bannick, EM, Wielopolski, PA, Pattynama, PM, Muinck Keizer-Schrama, S, Helbing, WA. Aortic distensibility and dimensions and the effects of growth hormone treatment in Turner syndrome. Am J Cardiol 2006; 97: 16441649.Google Scholar
28. 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’s syndrome. J Clin Endrocrinol Metab 2004; 89: 59665971.Google Scholar
29. Lacro, RV, Guey, LT, Dietz, HC, et al. Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy. Am Heart J 2013; 165: 828835.Google Scholar
30. Tierney, S, Levine, JC, Chen, S, et al. Echocardiographic methods, quality review, and measurement accuracy in a randomized multicenter clinical trial of Marfan syndrome. J Am Soc Echocardiogr 2013; 26: 657666.Google Scholar
31. Onis, M, Onyango, AW, Borghi, E, Siyam, A, Nishida, C, Siekmann, J. Development of a WHO growth reference for school-aged children and adolescents. Bulletin of the World Health Organization 2007; 85: 660667.Google Scholar