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Published in: Journal of Cardiovascular Magnetic Resonance 1/2011

Open Access 01-12-2011 | Research

Dilation of the ascending aorta in Turner syndrome - a prospective cardiovascular magnetic resonance study

Authors: Kristian H Mortensen, Britta E Hjerrild, Kirstine Stochholm, Niels H Andersen, Keld Ejvind Sørensen, Erik Lundorf, Arne Hørlyck, Erik M Pedersen, Jens S Christiansen, Claus H Gravholt

Published in: Journal of Cardiovascular Magnetic Resonance | Issue 1/2011

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Abstract

Background

The risk of aortic dissection is 100-fold increased in Turner syndrome (TS). Unfortunately, risk stratification is inadequate due to a lack of insight into the natural course of the syndrome-associated aortopathy. Therefore, this study aimed to prospectively assess aortic dimensions in TS.

Methods

Eighty adult TS patients were examined twice with a mean follow-up of 2.4 ± 0.4 years, and 67 healthy age and gender-matched controls were examined once. Aortic dimensions were measured at nine predefined positions using 3D, non-contrast and free-breathing cardiovascular magnetic resonance. Transthoracic echocardiography and 24-hour ambulatory blood pressure were also performed.

Results

At baseline, aortic diameters (body surface area indexed) were larger at all positions in TS. Aortic dilation was more prevalent at all positions excluding the distal transverse aortic arch. Aortic diameter increased in the aortic sinus, at the sinotubular junction and in the mid-ascending aorta with growth rates of 0.1 - 0.4 mm/year. Aortic diameters at all other positions were unchanged. The bicuspid aortic valve conferred higher aortic sinus growth rates (p < 0.05). No other predictors of aortic growth were identified.

Conclusion

A general aortopathy is present in TS with enlargement of the ascending aorta, which is accelerated in the presence of a bicuspid aortic valve.
Appendix
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Literature
1.
go back to reference 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: 4735-4742. 10.1210/jc.2008-1049.CrossRefPubMed 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: 4735-4742. 10.1210/jc.2008-1049.CrossRefPubMed
2.
go back to reference 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: 3897-3902. 10.1210/jc.2006-0558.CrossRefPubMed 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: 3897-3902. 10.1210/jc.2006-0558.CrossRefPubMed
4.
go back to reference 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: 745-749. 10.1136/jmg.2007.052019.PubMedCentralCrossRefPubMed 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: 745-749. 10.1136/jmg.2007.052019.PubMedCentralCrossRefPubMed
5.
go back to reference Gravholt CH, Landin-Wilhelmsen K, Stochholm K, Hjerrild BE, Ledet T, Djurhuus CB, Sylvén L, Baandrup U, Kristensen BØ, Christiansen JS: Clinical and epidemiological description of aortic dissection in Turner's syndrome. Cardiol Young. 2006, 16: 430-436. 10.1017/S1047951106000928.CrossRefPubMed Gravholt CH, Landin-Wilhelmsen K, Stochholm K, Hjerrild BE, Ledet T, Djurhuus CB, Sylvén L, Baandrup U, Kristensen BØ, Christiansen JS: Clinical and epidemiological description of aortic dissection in Turner's syndrome. Cardiol Young. 2006, 16: 430-436. 10.1017/S1047951106000928.CrossRefPubMed
6.
go back to reference Hjerrild BE, Mortensen KH, Sorensen KE, Pedersen EM, Andersen NH, Lundorf E, Hansen KW, Hørlyck A, Hager A, Christiansen JS, Gravholt CH: Thoracic aortopathy in Turner syndrome and the influence of bicuspid aortic valves and blood pressure: a CMR study. J Cardiovasc Magn Reson. 2010, 12: 12-10.1186/1532-429X-12-12.PubMedCentralCrossRefPubMed Hjerrild BE, Mortensen KH, Sorensen KE, Pedersen EM, Andersen NH, Lundorf E, Hansen KW, Hørlyck A, Hager A, Christiansen JS, Gravholt CH: Thoracic aortopathy in Turner syndrome and the influence of bicuspid aortic valves and blood pressure: a CMR study. J Cardiovasc Magn Reson. 2010, 12: 12-10.1186/1532-429X-12-12.PubMedCentralCrossRefPubMed
7.
go back to reference Matura LA, Ho VB, Rosing DR, Bondy CA: Aortic dilatation and dissection in Turner syndrome. Circulation. 2007, 116: 1663-1670. 10.1161/CIRCULATIONAHA.106.685487.CrossRefPubMed Matura LA, Ho VB, Rosing DR, Bondy CA: Aortic dilatation and dissection in Turner syndrome. Circulation. 2007, 116: 1663-1670. 10.1161/CIRCULATIONAHA.106.685487.CrossRefPubMed
8.
go back to reference Lopez L, Arheart KL, Colan SD, Stein NS, Lopez-Mitnik G, Lin AE, Reller MD, Ventura R, Silberbach M: Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics. 2008, 121: e1622-e1627. 10.1542/peds.2007-2807.CrossRefPubMed Lopez L, Arheart KL, Colan SD, Stein NS, Lopez-Mitnik G, Lin AE, Reller MD, Ventura R, Silberbach M: Turner syndrome is an independent risk factor for aortic dilation in the young. Pediatrics. 2008, 121: e1622-e1627. 10.1542/peds.2007-2807.CrossRefPubMed
9.
go back to reference 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 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
10.
go back to reference 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.x.CrossRef 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.x.CrossRef
11.
go back to reference 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, 20: 307-313. 10.1016/j.echo.2006.08.028.CrossRefPubMed 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, 20: 307-313. 10.1016/j.echo.2006.08.028.CrossRefPubMed
12.
go back to reference 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. 10.1016/j.jacc.2008.02.035.CrossRefPubMed 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. 10.1016/j.jacc.2008.02.035.CrossRefPubMed
13.
go back to reference 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. 10.1210/jc.2004-1090.CrossRefPubMed 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. 10.1210/jc.2004-1090.CrossRefPubMed
14.
go back to reference Mortensen KH, Hjerrild BE, Andersen NH, Sorensen KE, Horlyck 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, 1-10. Mortensen KH, Hjerrild BE, Andersen NH, Sorensen KE, Horlyck 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, 1-10.
15.
go back to reference Sorensen TS, Korperich H, Greil GF, Eichhorn J, Barth P, Meyer H, Pedersen EM, Beerbaum P: Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation. 2004, 110: 163-169. 10.1161/01.CIR.0000134282.35183.AD.CrossRefPubMed Sorensen TS, Korperich H, Greil GF, Eichhorn J, Barth P, Meyer H, Pedersen EM, Beerbaum P: Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation. 2004, 110: 163-169. 10.1161/01.CIR.0000134282.35183.AD.CrossRefPubMed
16.
go back to reference Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, Shinn JA, Svensson LG, Williams DM, American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American College of Radiology; American Stroke Association; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Interventional Radiology; Society of Thoracic Surgeons; Society for Vascular Medicine: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010, 121: e266-e369.CrossRefPubMed Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, Shinn JA, Svensson LG, Williams DM, American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American College of Radiology; American Stroke Association; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Interventional Radiology; Society of Thoracic Surgeons; Society for Vascular Medicine: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010, 121: e266-e369.CrossRefPubMed
17.
go back to reference Schaefer BM, Lewin MB, Stout KK, Gill E, Prueitt A, Byers PH, Otto CM: The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape. Heart. 2008, 94: 1634-1638. 10.1136/hrt.2007.132092.CrossRefPubMed Schaefer BM, Lewin MB, Stout KK, Gill E, Prueitt A, Byers PH, Otto CM: The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape. Heart. 2008, 94: 1634-1638. 10.1136/hrt.2007.132092.CrossRefPubMed
18.
go back to reference American College of Cardiology/American Heart Association Task Force on Practice Guidelines; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Thoracic Surgeons, Bonow RO, Carabello BA, Kanu C, de Leon AC, Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O'Gara PT, O'Rourke RA, Otto CM, Shah PM, Shanewise JS, Smith SC, Jacobs AK, Adams CD, Anderson JL, Antman EM, Faxon DP, Fuster V, Halperin JL, Hiratzka LF, Hunt SA, Lytle BW, Nishimura R, Page RL, Riegel B: ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation. 2006, 114: e84-231. 10.1161/CIRCULATIONAHA.106.176857.CrossRef American College of Cardiology/American Heart Association Task Force on Practice Guidelines; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Thoracic Surgeons, Bonow RO, Carabello BA, Kanu C, de Leon AC, Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O'Gara PT, O'Rourke RA, Otto CM, Shah PM, Shanewise JS, Smith SC, Jacobs AK, Adams CD, Anderson JL, Antman EM, Faxon DP, Fuster V, Halperin JL, Hiratzka LF, Hunt SA, Lytle BW, Nishimura R, Page RL, Riegel B: ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation. 2006, 114: e84-231. 10.1161/CIRCULATIONAHA.106.176857.CrossRef
19.
go back to reference Bland JM, Altman DG: Measuring agreement in method comparison studies. Stat Methods Med Res. 1999, 8: 135-160. 10.1191/096228099673819272.CrossRefPubMed Bland JM, Altman DG: Measuring agreement in method comparison studies. Stat Methods Med Res. 1999, 8: 135-160. 10.1191/096228099673819272.CrossRefPubMed
20.
go back to reference 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. 10.1161/01.CIR.0000142290.35842.B0.CrossRefPubMed 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. 10.1161/01.CIR.0000142290.35842.B0.CrossRefPubMed
21.
go back to reference Burman ED, Keegan J, Kilner PJ: Aortic root measurement by cardiovascular magnetic resonance: specification of planes and lines of measurement and corresponding normal values. Circ Cardiovasc Imaging. 2008, 1: 104-113. 10.1161/CIRCIMAGING.108.768911.CrossRefPubMed Burman ED, Keegan J, Kilner PJ: Aortic root measurement by cardiovascular magnetic resonance: specification of planes and lines of measurement and corresponding normal values. Circ Cardiovasc Imaging. 2008, 1: 104-113. 10.1161/CIRCIMAGING.108.768911.CrossRefPubMed
22.
go back to reference Davies RR, Kaple RK, Mandapati D, Gallo A, Botta DM, Elefteriades JA, Coady MA: Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg. 2007, 83: 1338-1344. 10.1016/j.athoracsur.2006.10.074.CrossRefPubMed Davies RR, Kaple RK, Mandapati D, Gallo A, Botta DM, Elefteriades JA, Coady MA: Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg. 2007, 83: 1338-1344. 10.1016/j.athoracsur.2006.10.074.CrossRefPubMed
23.
go back to reference Hjerrild BE, Mortensen KH, Gravholt CH: Turner syndrome and clinical treatment. Br Med Bull. 2008, 86: 77-93. 10.1093/bmb/ldn015.CrossRefPubMed Hjerrild BE, Mortensen KH, Gravholt CH: Turner syndrome and clinical treatment. Br Med Bull. 2008, 86: 77-93. 10.1093/bmb/ldn015.CrossRefPubMed
Metadata
Title
Dilation of the ascending aorta in Turner syndrome - a prospective cardiovascular magnetic resonance study
Authors
Kristian H Mortensen
Britta E Hjerrild
Kirstine Stochholm
Niels H Andersen
Keld Ejvind Sørensen
Erik Lundorf
Arne Hørlyck
Erik M Pedersen
Jens S Christiansen
Claus H Gravholt
Publication date
01-12-2011
Publisher
BioMed Central
Published in
Journal of Cardiovascular Magnetic Resonance / Issue 1/2011
Electronic ISSN: 1532-429X
DOI
https://doi.org/10.1186/1532-429X-13-24

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