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Journal of Cardiovascular Magnetic Resonance
Published in: Journal of Cardiovascular Magnetic Resonance 1/2017

Open Access 01-12-2017 | Research

Proximal aortic stiffening in Turner patients may be present before dilation can be detected: a segmental functional MRI study

Authors: Daniel G. H. Devos, Katya De Groote, Danilo Babin, Laurent Demulier, Yves Taeymans, Jos J. Westenberg, Luc Van Bortel, Patrick Segers, Eric Achten, Jean De Schepper, Ernst Rietzschel

Published in: Journal of Cardiovascular Magnetic Resonance | Issue 1/2017

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Abstract

Background

To study segmental structural and functional aortic properties in Turner syndrome (TS) patients. Aortic abnormalities contribute to increased morbidity and mortality of women with Turner syndrome. Cardiovascular magnetic resonance (CMR) allows segmental study of aortic elastic properties.

Method

We performed Pulse Wave Velocity (PWV) and distensibility measurements using CMR of the thoracic and abdominal aorta in 55 TS-patients, aged 13-59y, and in a control population (n = 38;12-58y). We investigated the contribution of TS on aortic stiffness in our entire cohort, in bicuspid (BAV) versus tricuspid (TAV) aortic valve-morphology subgroups, and in the younger and older subgroups.

Results

Differences in aortic properties were only seen at the most proximal aortic level. BAV Turner patients had significantly higher PWV, compared to TAV Turner (p = 0.014), who in turn had significantly higher PWV compared to controls (p = 0.010). BAV Turner patients had significantly larger ascending aortic (AA) luminal area and lower AA distensibility compared to both controls (all p < 0.01) and TAV Turner patients. TAV Turner had similar AA luminal areas and AA distensibility compared to Controls. Functional changes are present in younger and older Turner subjects, whereas ascending aortic dilation is prominent in older Turner patients. Clinically relevant dilatation (TAV and BAV) was associated with reduced distensibility.

Conclusion

Aortic stiffening and dilation in TS affects the proximal aorta, and is more pronounced, although not exclusively, in BAV TS patients.
Functional abnormalities are present at an early age, suggesting an aortic wall disease inherent to the TS. Whether this increased stiffness at young age can predict later dilatation needs to be studied longitudinally.
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Literature
1.
Stochholm K, Juul S, Juel K, Naeraa RW, Gravholt C. Prevalence, incidence, diagnostic delay, and mortality in turner syndrome. J Clin Endocrinol Metabolism. 2006;91(10):3897–902.CrossRef
2.
Sachdev V, Matura LA, Sidenko S, Ho VB, Arai AE, Rosing DR, et al. Aortic valve disease in turner syndrome. J Am Coll Cardiol. 2008;51(19):1904–9.CrossRefPubMed
3.
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;71:433–6.CrossRefPubMedPubMedCentral
4.
Ho VB, Bakalov VK, Cooley M, Van PL, Hood MN, Burklow TR, et al. Major vascular anomalies in turner syndrome: prevalence and magnetic resonance angiographic features. Circulation. 2004;110(12):1694–700.CrossRefPubMed
5.
Mazzanti L, Prandstraller D, Fattori R, Lovato L, Cicognani A. Monitoring of congenital heart disease (CHD) and aortic dilatation in Turner syndrome: Italian experience. Int Congress Ser. 2006;1298(Complete):123–30.CrossRef
6.
Mazzanti L, Cacciari E. Congenital heart disease in patients with Turner’s syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediatr. 1998;5:688–92.CrossRef
7.
Mortensen KH, Hjerrild BE, Andersen NH, Sorensen KE, Horlyck A, Edersen EM, et al. Abnormalities of the major intrathoracic arteries in Turner syndrome as revealed by magnetic resonance imaging. Cardiol Young. 2010;20(02):191–200.CrossRefPubMed
8.
Prandstraller D, Mazzanti L, Picchio F, Magnani C, Bergamaschi R, Perri A, et al. Turner’s syndrome: cardiologic profile according to the different chromosomal patterns and long-term clinical follow-up of 136 nonpreselected patients. Pediatr Cardiol. 1999;20(2):108–12.CrossRefPubMed
9.
Völkl T, Degenhardt K, Koch A, Simm D, Dörr H, Singer H. Cardiovascular anomalies in children and young adults with Ullrich-Turner syndrome the Erlangen experience. Clin Cardiol. 2005;28(2):88–92.CrossRefPubMed
10.
Dawson-Falk K, Wright A, Bakker B, Pitlick P, Wilson D, Rosenfeld R. Cardiovascular evaluation in Turner syndrome: utility of MR imaging. Australas Radiol. 1992;36(3):204–9.CrossRefPubMed
11.
Kim HK, Gottliebson W, Hor K, Backeljauw P, Gutmark-Little I, Salisbury SR, et al. Cardiovascular anomalies in turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population. Am J Roentgenol. 2011;196(2):454–60.CrossRef
12.
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 Metabolism. 2008;93(12):4735–42.CrossRef
13.
Gravholt CH, Juul S, Naeraa RW, Hansen J. Morbidity in turner syndrome. J Clin Epidemiol. 1998;51(2):147–58.CrossRefPubMed
14.
Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey Jr DE, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. J Am Coll Cardiol. 2010;55(14):e27–e129.CrossRefPubMed
15.
Mortensen K, Erlandsen M, Andersen N, Gravholt C. Prediction of aortic dilation in Turner syndrome - enhancing the use of serial cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2013;15(1):47–58.CrossRefPubMedPubMedCentral
16.
Segers P, De Backer J, Devos D, RABBEN SI, Gillebert T, Van Bortel L, et al. Aortic reflection coefficients and their association with global indexes of wave reflection in healthy controls and patients with Marfan’s syndrome. Am J Physiol Heart Circ Physiol. 2006;290(6):2385.CrossRef
17.
Blacher J, Guerin AP, Pannier B, Marchais SJ, Safar ME, London GM. Impact of aortic stiffness on survival in end-stage renal disease. Circulation. 1999;99(18):2434–9.CrossRefPubMed
18.
Laurent S, Boutouyrie P, Asmar R, Gautier I, Laloux B, Guize L, et al. Aortic stiffness is an independent predictor of all-cause and cardiovascular mortality in hypertensive patients. Hypertension. 2001;37(5):1236–41.CrossRefPubMed
19.
Mattace-Raso FUS, van der Cammen TJM, Hofman A, van Popele NM, Bos ML, Schalekamp MADH, et al. Arterial stiffness and risk of coronary heart disease and stroke: the Rotterdam study. Circulation. 2006;113(5):657–63.CrossRefPubMed
20.
Meaume S, Benetos A, Henry OF, Rudnichi A, Safar ME. Aortic pulse wave velocity predicts cardiovascular mortality in subjects >70 years of age. Arterioscler Thromb Vasc Biol. 2001;21(12):2046–50.CrossRefPubMed
21.
Sutton-Tyrrell K, Najjar SS, Boudreau RM, Venkitachalam L, Kupelian V, Simonsick EM, et al. Elevated aortic pulse wave velocity, a marker of arterial stiffness, predicts cardiovascular events in well-functioning older adults. Circulation. 2005;111(25):3384–90.CrossRefPubMed
22.
Ben-Shlomo Y, Spears M, Boustred C, May M, Anderson SG, Benjamin EJ, et al. Aortic pulse wave velocity improves cardiovascular event prediction: an individual participant meta-analysis of prospective-observational data from 17,635 subjects. J Am Coll Cardiol. 2014;63(7):636–46.CrossRefPubMed
23.
Meloni A, Zymeski H, Pepe A, Lombardi M, Wood JC. Robust estimation of pulse wave transit time using group delay. J Magn Reson Imaging. 2014;39(3):550–8.CrossRefPubMed
24.
van Popele NM, Grobbee DE, Bots ML, Asmar R, Topouchian J, Reneman RS, et al. Association between arterial stiffness and atherosclerosis: the Rotterdam study. Stroke. 2001;32(2):454–60.CrossRefPubMed
25.
Vlachopoulos C, Aznaouridis K, Stefanadis C. Prediction of cardiovascular events and all-cause mortality with arterial stiffness: a systematic review and meta-analysis. J Am Coll Cardiol. 2010;55(13):1318–27.CrossRefPubMed
26.
Devos DGH, Rietzschel E, Heyse C, Vandemaele P, Van Bortel L, Babin D, et al. MR pulse wave velocity increases with age faster in the thoracic aorta than in the abdominal aorta. J Magn Reson Imaging. 2015;41(3):765–72.CrossRefPubMed
27.
Mosteller RD. Simplified calculation of body-surface area. N Engl J Med. 1987;317(17):1098.PubMed
28.
Matura LA, Ho VB, Rosing DR, Bondy CA. Aortic dilatation and dissection in turner syndrome. Circulation. 2007;116(15):1663–70.CrossRefPubMed
29.
Grotenhuis HB, Westenberg JJ, Steendijk P, van der Geest RJ, Ottenkamp J, Bax JJ, et al. Validation and reproducibility of aortic pulse wave velocity as assessed with velocity-encoded MRI. J Magn Reson Imaging. 2009;30(3):521–6.CrossRefPubMed
30.
Mortensen KH, Hjerrild BE, Stochholm K, Andersen NH, Sorensen KE, Lundorf E, et al. Dilation of the ascending aorta in Turner syndrome - a prospective cardiovascular magnetic resonance study. J Cardiovasc Magn Reson. 2011;13:24–33.CrossRefPubMedPubMedCentral
31.
Ostberg JE, Brookes JAS, 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 Metabol. 2004;89(12):5966–71.CrossRef
32.
Olivieri LJ, Baba RY, Arai AE, Bandettini WP, Rosing DR, Bakalov V, et al. Spectrum of aortic valve abnormalities associated with aortic dilation across age groups in turner syndrome. Circ Cardiovasc Imaging. 2013;6:1018–23.CrossRefPubMedPubMedCentral
33.
Rogers WJ, Hu YL, Coast D, Vido DA, Kramer CM, Pyeritz RE, et al. Age-associated changes in regional aortic pulse wave velocity. J Am Coll Cardiol. 2001;38(4):1123–9.CrossRefPubMed
34.
Lawson SA, Urbina EM, Gutmark-Little I, Khoury PR, Gao Z, Backeljauw PF. Vasculopathy in the young turner syndrome population. J Clin Endocrinol Metabolism. 2014;99(10):E2039–45.CrossRef
35.
Baguet JP, Douchin S, Pierre H, Rossignol AM, Bost M, Mallion JM. Structural and functional abnormalities of large arteries in the Turner syndrome. Heart. 2005;91(11):1442–6.CrossRefPubMedPubMedCentral
36.
De Groote K, Devos D, Van Herck K, Demulier L, Buysse W, De Schepper J, et al. Abnormal aortic arch morphology in Turner syndrome patients is a risk factor for hypertension. Heart Vessels. 2015;30(5):618–25.CrossRefPubMed
37.
Sybert V. Cardiovascular malformations and complications in Turner syndrome. Pediatrics. 1998;101(1):E11.CrossRefPubMed
38.
Gravholt CH, Landin-Wilhelmsen K, Stochholm K, Eilersen Hjerrild B, Ledet T, Born Djurhuus C, et al. Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young. 2006;16(05):430–6.CrossRefPubMed
39.
Meszaros I, Morocz J, Szlavi J, Schmidt J, Tornoci L, Nagy L, et al. Epidemiology and clinicopathology of aortic dissection: A population-based longitudinal study over 27 years. Chest. 2000;117(5):1271–8.CrossRefPubMed
40.
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(12):745–9.CrossRefPubMedPubMedCentral
41.
Hagan PG, Nienaber CA, Isselbacher EM. The international registry of acute aortic dissection (irad): New insights into an old disease. JAMA. 2000;283(7):897–903.CrossRefPubMed
42.
43.
Bondy CA. Care of girls and women with Turner syndrome: A guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab. 2007;92:10–25.CrossRefPubMed
44.
Turtle EJ, Sule AA, Webb DJ, Bath LE. Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations. Arch Dis Child. 2015;100(7):662–6.CrossRefPubMed
45.
Mortensen KH, Hansen KW, Erlandsen M, Christiansen JS, Gravholt CH. Ambulatory arterial stiffness index in Turner syndrome: the impact of sex hormone replacement therapy. Horm Res. 2009;72:184–9.CrossRefPubMed
46.
Fielden SW, Fornwalt BK, Jerosch-Herold M, Eisner RL, Stillman AE, Oshinski JN. A new method for the determination of aortic pulse wave velocity using cross-correlation on 2D PCMR velocity data. J Magn Reson Imaging. 2008;27(6):1382–7.CrossRefPubMed
47.
Westenberg JJM, de Roos A, Grotenhuis HB, Steendijk P, Hendriksen D, van den Boogaard PJ, et al. Improved aortic pulse wave velocity assessment from multislice two-directional in-plane velocity-encoded magnetic resonance imaging. J Magn Reson Imaging. 2010;32(5):1086–94.CrossRefPubMed
48.
Mohiaddin RH, Firmin DN, Longmore DB. Age-related changes of human aortic flow wave velocity measured noninvasively by magnetic resonance imaging. J Appl Physiol. 1993;74(1):492.PubMed
49.
Dorniak K, Heiberg E, Hellmann M, Rawicz-Zegrzda D, Wesierska M, Galaska R, et al. Required temporal resolution for accurate thoracic aortic pulse wave velocity measurements by phase-contrast magnetic resonance imaging and comparison with clinical standard applanation tonometry. BMC Cardiovasc Disord. 2016;16(1):110.CrossRefPubMedPubMedCentral
50.
Bargiotas I, Mousseaux E, Yu WC, Venkatesh BA, Bollache E, de Cesare A, et al. Estimation of aortic pulse wave transit time in cardiovascular magnetic resonance using complex wavelet cross-spectrum analysis. J Cardiovasc Magn Reson. 2015;17(1):65.CrossRefPubMedPubMedCentral
51.
Bell V, Mitchell WA, Sigurdsson S, Westenberg JJM, Gotal JD, Torjesen AA, et al. Longitudinal and circumferential strain of the proximal aorta. J Am Heart Assoc. 2014;3(6):e001536.CrossRefPubMedPubMedCentral
Metadata
Title
Proximal aortic stiffening in Turner patients may be present before dilation can be detected: a segmental functional MRI study
Authors
Daniel G. H. Devos
Katya De Groote
Danilo Babin
Laurent Demulier
Yves Taeymans
Jos J. Westenberg
Luc Van Bortel
Patrick Segers
Eric Achten
Jean De Schepper
Ernst Rietzschel
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Journal of Cardiovascular Magnetic Resonance / Issue 1/2017
Electronic ISSN: 1532-429X
DOI
https://doi.org/10.1186/s12968-017-0331-0

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