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

Daniel Devos (UGent) , Katya De Groote (UGent) , Danilo Babin (UGent) , Laurent Demulier (UGent) , Yvo Taeymans (UGent) , Jos J Westenberg, Lucas Van Bortel (UGent) , Patrick Segers (UGent) , Eric Achten (UGent) , Jean De Schepper (UGent) , et al.
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Organization
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.
Keywords
PULSE-WAVE VELOCITY, CARDIOVASCULAR MAGNETIC-RESONANCE, ARTERIAL STIFFNESS, TRANSIT-TIME, ALL-CAUSE, DISSECTION, MORTALITY, DISEASE, AGE, PREVALENCE

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MLA
Devos, Daniel, Katya De Groote, Danilo Babin, et al. “Proximal Aortic Stiffening in Turner Patients May Be Present Before Dilation Can Be Detected : a Segmental Functional MRI Study.” JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE 19 (2017): n. pag. Print.
APA
Devos, Daniel, De Groote, K., Babin, D., Demulier, L., Taeymans, Y., Westenberg, J. J., Van Bortel, L., et al. (2017). Proximal aortic stiffening in Turner patients may be present before dilation can be detected : a segmental functional MRI study. JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE, 19.
Chicago author-date
Devos, Daniel, Katya De Groote, Danilo Babin, Laurent Demulier, Yvo Taeymans, Jos J Westenberg, Lucas Van Bortel, et al. 2017. “Proximal Aortic Stiffening in Turner Patients May Be Present Before Dilation Can Be Detected : a Segmental Functional MRI Study.” Journal of Cardiovascular Magnetic Resonance 19.
Chicago author-date (all authors)
Devos, Daniel, Katya De Groote, Danilo Babin, Laurent Demulier, Yvo Taeymans, Jos J Westenberg, Lucas Van Bortel, Patrick Segers, Eric Achten, Jean De Schepper, and Ernst Rietzschel. 2017. “Proximal Aortic Stiffening in Turner Patients May Be Present Before Dilation Can Be Detected : a Segmental Functional MRI Study.” Journal of Cardiovascular Magnetic Resonance 19.
Vancouver
1.
Devos D, De Groote K, Babin D, Demulier L, Taeymans Y, Westenberg JJ, et al. Proximal aortic stiffening in Turner patients may be present before dilation can be detected : a segmental functional MRI study. JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE. 2017;19.
IEEE
[1]
D. Devos et al., “Proximal aortic stiffening in Turner patients may be present before dilation can be detected : a segmental functional MRI study,” JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE, vol. 19, 2017.
@article{8518112,
  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.},
  articleno    = {27},
  author       = {Devos, Daniel and De Groote, Katya and Babin, Danilo and Demulier, Laurent and Taeymans, Yvo and Westenberg, Jos J and Van Bortel, Lucas and Segers, Patrick and Achten, Eric and De Schepper, Jean and Rietzschel, Ernst},
  issn         = {1097-6647},
  journal      = {JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE},
  keywords     = {PULSE-WAVE VELOCITY,CARDIOVASCULAR MAGNETIC-RESONANCE,ARTERIAL STIFFNESS,TRANSIT-TIME,ALL-CAUSE,DISSECTION,MORTALITY,DISEASE,AGE,PREVALENCE},
  language     = {eng},
  pages        = {12},
  title        = {Proximal aortic stiffening in Turner patients may be present before dilation can be detected : a segmental functional MRI study},
  url          = {http://dx.doi.org/10.1186/s12968-017-0331-0},
  volume       = {19},
  year         = {2017},
}

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