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

Journal of Cardiovascular Magnetic Resonance

Research

Laurent Demulier¹  Yves Taeymans¹  Ernst Rietzschel¹  Jos J. Westenberg²  Eric Achten³  Daniel G. H. Devos³  Luc Van Bortel⁴  Patrick Segers⁵  Katya De Groote⁶  Jean De Schepper⁷  Danilo Babin⁸ 

[1] Department of Cardiology, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium;Department of Radiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands;Department of Radiology, MRI (-1K12), Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium;Heymans Institute of Pharmacology, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium;IBiTech-bioMMeda, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium;Pediatric Cardiology, Department of Pediatrics and Turner Clinic, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium;Pediatric Endocrinology, Department of Pediatrics and Turner Clinic, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium;Telecommunications and Information Processing, TELIN-IPI-iMinds, Faculty of Engineering and Architecture, Ghent University, Sint-Pietersnieuwstraat 41, 9000, Ghent, Belgium;

关键词: Cardiovascular Magnetic Resonance;    Pulse Wave Velocity;    Turner Syndrome;    Bicuspid Aortic Valve;    Estrogen Replacement Therapy;   

DOI  :  10.1186/s12968-017-0331-0

 received in 2016-11-08, accepted in 2017-01-20,  发布年份 2017

来源: Springer

【 摘 要 】

BackgroundTo 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.MethodWe 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.ResultsDifferences 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.ConclusionAortic 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.

【 授权许可】

CC BY   
© The Author(s). 2017