Thoracic aortopathy in Turner syndrome and the influence of bicuspid aortic valves and blood pressure: a CMR study.
BACKGROUND
To investigate aortic dimensions in women with Turner syndrome (TS) in relation to aortic valve morphology, blood pressure, karyotype, and clinical characteristics.


RESULTS
A cross sectional study of 102 women with TS (mean age 37.7; 18-62 years) examined by cardiovascular magnetic resonance (CMR- successful in 95), echocardiography, and 24-hour ambulatory blood pressure. Aortic diameters were measured by CMR at 8 positions along the thoracic aorta. Twenty-four healthy females were recruited as controls. In TS, aortic dilatation was present at one or more positions in 22 (23%). Aortic diameter in women with TS and bicuspid aortic valve was significantly larger than in TS with tricuspid valves in both the ascending (32.4 +/- 6.7 vs. 26.0 +/- 4.4 mm; p < 0.001) and descending (21.4 +/- 3.5 vs. 18.8 +/- 2.4 mm; p < 0.001) aorta. Aortic diameter correlated to age (R = 0.2 - 0.5; p < 0.01), blood pressure (R = 0.4; p < 0.05), a history of coarctation (R = 0.3; p = 0.01) and bicuspid aortic valve (R = 0.2-0.5; p < 0.05). Body surface area only correlated with descending aortic diameter (R = 0.23; p = 0.024).


CONCLUSIONS
Aortic dilatation was present in 23% of adult TS women, where aortic valve morphology, age and blood pressure were major determinants of the aortic diameter.
Hjerrild
Britta E
BE
Medical Department M (Endocrinology and Diabetes) and Medical Research Laboratories, Aarhus Sygehus NBG, Aarhus University Hospital, DK-8000 Aarhus C, Denmark. hjerrild@dadlnet.dk
Mortensen
Kristian H
KH
Sørensen
Keld E
KE
Pedersen
Erik M
EM
Andersen
Niels H
NH
Lundorf
Erik
E
Hansen
Klavs W
KW
Hørlyck
Arne
A
Hager
Alfred
A
Christiansen
Jens S
JS
Gravholt
Claus H
CH
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001013:Aorta, Thoracic / Q000473:pathology* / Q000503:physiopathology; D001017:Aortic Coarctation / Q000150:complications; D001018:Aortic Diseases / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D001021:Aortic Valve / Q000002:abnormalities* / Q000503:physiopathology; D001794:Blood Pressure; D018660:Blood Pressure Monitoring, Ambulatory; D001830:Body Surface Area; D016022:Case-Control Studies; D016009:Chi-Square Distribution; D003430:Cross-Sectional Studies; D004108:Dilatation, Pathologic; D004452:Echocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D016014:Linear Models; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D018570:Risk Assessment; D012307:Risk Factors; D014424:Turner Syndrome / Q000150:complications* / Q000503:physiopathology; D055815:Young Adult