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Published in: Pediatric Cardiology 4/2010

01-05-2010 | Original Article

Aortic Dimensions in Girls and Young Women with Turner Syndrome: A Magnetic Resonance Imaging Study

Authors: Line Cleemann, Kristian H. Mortensen, Kirsten Holm, Heidi Smedegaard, Sven O. Skouby, Steen B. Wieslander, Anne-Mette Leffers, Per Leth-Espensen, Erik Morre Pedersen, Claus H. Gravholt

Published in: Pediatric Cardiology | Issue 4/2010

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Abstract

This study aimed to determine the dimensions of the thoracic aorta and the predictors of aortic dimensions in girls and young women with Turner syndrome (TS). A cross-sectional study was performed at a secondary care center. The study compared 41 TS patients with 50 healthy age-matched control subjects. The mean age of the patients was 17 ± 3.3 years. Magnetic resonance imaging was performed for all the patients. The thoracic aortic diameters of the patients were measured at nine positions. Adjustment for body surface area (BSA) was performed. The outcome for the patients was measured in terms of absolute and BSA-adjusted aortic dilation. In TS, both the absolute and the BSA-adjusted mean aortic diameters were smaller than or comparable with those of the control subjects. However, individual aortic dilation at one to four positions was found in four TS patients according to the uncorrected data and in five TS patients after BSA-adjustment. The aortic diameters correlated with height, weight, body mass index (BMI), and BSA at all positions (R = 0.34–0.60; all p < 0.04). The diameters of the aortic arch and the descending aorta correlated with a history of aortic coarctation (R = 0.35–0.52; p < 0.03). The presence of bicuspid aortic valves correlated at the descending part of the aorta (R = 0.38; p < 0.03). The mean thoracic aortic dimensions were not enlarged in girls or young TS patients. The BSA predicted aortic size at all positions. The prevalence of aortic dilation and aneurysm was lower in this population of girls and younger women with TS than in older TS populations.
Literature
1.
go back to reference Bondy CA (2007) Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab 92:10–25CrossRefPubMed Bondy CA (2007) Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab 92:10–25CrossRefPubMed
2.
go back to reference Bondy CA (2007) Heart disease in Turner syndrome. Minerva Endocrinol 32:245–261PubMed Bondy CA (2007) Heart disease in Turner syndrome. Minerva Endocrinol 32:245–261PubMed
3.
go back to reference Bondy CA (2008) Congenital cardiovascular disease in Turner syndrome. Congenit Heart Dis 3:2–15PubMed Bondy CA (2008) Congenital cardiovascular disease in Turner syndrome. Congenit Heart Dis 3:2–15PubMed
4.
go back to reference Chalard F, Ferey S, Teinturier C, Kalifa G (2005) Aortic dilatation in Turner syndrome: the role of MRI in early recognition. Pediatr Radiol 35:323–326CrossRefPubMed Chalard F, Ferey S, Teinturier C, Kalifa G (2005) Aortic dilatation in Turner syndrome: the role of MRI in early recognition. Pediatr Radiol 35:323–326CrossRefPubMed
5.
go back to reference Dawson-Falk KL, Wright AM, Bakker B, Pitlick PT, Wilson DM, Rosenfeld RG (1992) Cardiovascular evaluation in Turner syndrome: utility of MR imaging. Australas Radiol 36:204–209CrossRefPubMed Dawson-Falk KL, Wright AM, Bakker B, Pitlick PT, Wilson DM, Rosenfeld RG (1992) Cardiovascular evaluation in Turner syndrome: utility of MR imaging. Australas Radiol 36:204–209CrossRefPubMed
6.
go back to reference 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 54:69–73CrossRefPubMed 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 54:69–73CrossRefPubMed
7.
go back to reference Gleason TG (2005) Heritable disorders predisposing to aortic dissection. Semin Thorac Cardiovasc Surg 17:274–281CrossRefPubMed Gleason TG (2005) Heritable disorders predisposing to aortic dissection. Semin Thorac Cardiovasc Surg 17:274–281CrossRefPubMed
8.
go back to reference Gotzsche CO, Krag-Olsen B, Nielsen J, Sorensen KE, Kristensen BO (1994) Prevalence of cardiovascular malformations and association with karyotypes in Turner’s syndrome. Arch Dis Child 71:433–436CrossRefPubMed Gotzsche CO, Krag-Olsen B, Nielsen J, Sorensen KE, Kristensen BO (1994) Prevalence of cardiovascular malformations and association with karyotypes in Turner’s syndrome. Arch Dis Child 71:433–436CrossRefPubMed
9.
go back to reference Gravholt CH, Landin-Wilhelmsen K, Stochholm K et al (2006) Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young 16:430–436CrossRefPubMed Gravholt CH, Landin-Wilhelmsen K, Stochholm K et al (2006) Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young 16:430–436CrossRefPubMed
10.
go back to reference Hager A, Kaemmerer H, Rapp-Bernhardt U et al (2002) Diameters of the thoracic aorta throughout life as measured with helical computed tomography. J Thorac Cardiovasc Surg 123:1060–1066CrossRefPubMed Hager A, Kaemmerer H, Rapp-Bernhardt U et al (2002) Diameters of the thoracic aorta throughout life as measured with helical computed tomography. J Thorac Cardiovasc Surg 123:1060–1066CrossRefPubMed
11.
go back to reference Januzzi JL, Eagle KA, Cooper JV et al (2005) Acute aortic dissection presenting with congestive heart failure: results from the International Registry of Acute Aortic Dissection. J Am Coll Cardiol 46:733–735CrossRefPubMed Januzzi JL, Eagle KA, Cooper JV et al (2005) Acute aortic dissection presenting with congestive heart failure: results from the International Registry of Acute Aortic Dissection. J Am Coll Cardiol 46:733–735CrossRefPubMed
12.
go back to reference Lanzarini L, Larizza D, Prete G, Calcaterra V, Klersy C (2007) Prospective evaluation of aortic dimensions in Turner syndrome: a 2-dimensional echocardiographic study. J Am Soc Echocardiogr 20:307–313CrossRefPubMed Lanzarini L, Larizza D, Prete G, Calcaterra V, Klersy C (2007) Prospective evaluation of aortic dimensions in Turner syndrome: a 2-dimensional echocardiographic study. J Am Soc Echocardiogr 20:307–313CrossRefPubMed
13.
go back to reference Lanzarini L, Larizza D, Prete G et al (2007) Aortic dimensions in Turner’s syndrome: two-dimensional echocardiography versus magnetic resonance imaging. J Cardiovasc Med Hagerstown 8:428–437PubMed Lanzarini L, Larizza D, Prete G et al (2007) Aortic dimensions in Turner’s syndrome: two-dimensional echocardiography versus magnetic resonance imaging. J Cardiovasc Med Hagerstown 8:428–437PubMed
14.
go back to reference Larson EW, Edwards WD (1984) Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol 53:849–855CrossRefPubMed Larson EW, Edwards WD (1984) Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol 53:849–855CrossRefPubMed
15.
go back to reference Lin AE, Lippe B, Rosenfeld RG (1998) Further delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome. Pediatrics 102:e12CrossRefPubMed Lin AE, Lippe B, Rosenfeld RG (1998) Further delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome. Pediatrics 102:e12CrossRefPubMed
16.
go back to reference Loeys B, De PA (2008) New insights in the pathogenesis of aortic aneurysms. Verh K Acad Geneeskd Belg 70:69–84PubMed Loeys B, De PA (2008) New insights in the pathogenesis of aortic aneurysms. Verh K Acad Geneeskd Belg 70:69–84PubMed
17.
go back to reference Lopez L, Arheart KL, Colan SD et al (2008) Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics 121:e1622–e1627CrossRefPubMed Lopez L, Arheart KL, Colan SD et al (2008) Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics 121:e1622–e1627CrossRefPubMed
18.
go back to reference Matura LA, Ho VB, Rosing DR, Bondy CA (2007) Aortic dilatation and dissection in Turner syndrome. Circulation 116(15):1663–1670CrossRefPubMed Matura LA, Ho VB, Rosing DR, Bondy CA (2007) Aortic dilatation and dissection in Turner syndrome. Circulation 116(15):1663–1670CrossRefPubMed
19.
go back to reference Mazzanti L, Cacciari E (1998) Congenital heart disease in patients with Turner’s syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediatr 133:688–692PubMed Mazzanti L, Cacciari E (1998) Congenital heart disease in patients with Turner’s syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediatr 133:688–692PubMed
20.
go back to reference Ostberg JE, Brookes JA, McCarthy C, Halcox J, Conway GS (2004) A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome. J Clin Endocrinol Metab 89:5966–5971CrossRefPubMed Ostberg JE, Brookes JA, McCarthy C, Halcox J, Conway GS (2004) A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome. J Clin Endocrinol Metab 89:5966–5971CrossRefPubMed
21.
go back to reference Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C, Conway GS (2005) Vasculopathy in Turner syndrome: arterial dilatation and intimal thickening without endothelial dysfunction. J Clin Endocrinol Metab 90:5161–5166CrossRefPubMed Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C, Conway GS (2005) Vasculopathy in Turner syndrome: arterial dilatation and intimal thickening without endothelial dysfunction. J Clin Endocrinol Metab 90:5161–5166CrossRefPubMed
22.
go back to reference Sachdev V, Matura LA, Sidenko S et al (2008) Aortic valve disease in Turner syndrome. J Am Coll Cardiol 51:1904–1909CrossRefPubMed Sachdev V, Matura LA, Sidenko S et al (2008) Aortic valve disease in Turner syndrome. J Am Coll Cardiol 51:1904–1909CrossRefPubMed
23.
go back to reference Schoemaker MJ, Swerdlow AJ, Higgins CD, Wright AF, Jacobs PA (2008) Cancer incidence in women with Turner syndrome in Great Britain: a national cohort study. Lancet Oncol 9:239–246CrossRefPubMed Schoemaker MJ, Swerdlow AJ, Higgins CD, Wright AF, Jacobs PA (2008) Cancer incidence in women with Turner syndrome in Great Britain: a national cohort study. Lancet Oncol 9:239–246CrossRefPubMed
24.
go back to reference Sorensen TS, Korperich H, Greil GF et al (2004) Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation 110:163–169CrossRefPubMed Sorensen TS, Korperich H, Greil GF et al (2004) Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation 110:163–169CrossRefPubMed
25.
go back to reference Stochholm K, Juul S, Juel K, Naeraa RW, Gravholt CH (2006) Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 91:3897–3902CrossRefPubMed Stochholm K, Juul S, Juel K, Naeraa RW, Gravholt CH (2006) Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 91:3897–3902CrossRefPubMed
26.
go back to reference Sybert VP (1998) Cardiovascular malformations and complications in Turner syndrome. Pediatrics 101:E11CrossRefPubMed Sybert VP (1998) Cardiovascular malformations and complications in Turner syndrome. Pediatrics 101:E11CrossRefPubMed
28.
go back to reference The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents (2004) Pediatrics 114(2 suppl 4):555–576 The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents (2004) Pediatrics 114(2 suppl 4):555–576
29.
go back to reference van den BJ, Bannink EM, Wielopolski PA, Pattynama PM, de Muinck Keizer-Schrama SM, Helbing WA (2006) Aortic distensibility and dimensions and the effects of growth hormone treatment in the turner syndrome. Am J Cardiol 97:1644–1649 van den BJ, Bannink EM, Wielopolski PA, Pattynama PM, de Muinck Keizer-Schrama SM, Helbing WA (2006) Aortic distensibility and dimensions and the effects of growth hormone treatment in the turner syndrome. Am J Cardiol 97:1644–1649
30.
go back to reference Von Tengg-Kobligk H, Weber TF, Rengier F et al (2008) Imaging modalities for the thoracic aorta. J Cardiovasc Surg (Torino) 49:429–447 Von Tengg-Kobligk H, Weber TF, Rengier F et al (2008) Imaging modalities for the thoracic aorta. J Cardiovasc Surg (Torino) 49:429–447
Metadata
Title
Aortic Dimensions in Girls and Young Women with Turner Syndrome: A Magnetic Resonance Imaging Study
Authors
Line Cleemann
Kristian H. Mortensen
Kirsten Holm
Heidi Smedegaard
Sven O. Skouby
Steen B. Wieslander
Anne-Mette Leffers
Per Leth-Espensen
Erik Morre Pedersen
Claus H. Gravholt
Publication date
01-05-2010
Publisher
Springer-Verlag
Published in
Pediatric Cardiology / Issue 4/2010
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-009-9626-8

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