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

• Published in: Journal of cardiovascular magnetic resonance 2011-04, Vol.13 (1), p.24-24, Article 24
• Main Authors: Mortensen, Kristian H, Hjerrild, Britta E, Stochholm, Kirstine, Andersen, Niels H, Sørensen, Keld Ejvind, Lundorf, Erik, Hørlyck, Arne, Pedersen, Erik M, Christiansen, Jens S, Gravholt, Claus H
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aorta - pathology; Aortic Aneurysm - diagnosis; Aortic Aneurysm - etiology; Aortic Aneurysm - pathology; Aortic Valve - abnormalities; Blood Pressure; Blood Pressure Monitoring, Ambulatory; Cardiovascular diseases; Case-Control Studies; Chi-Square Distribution; Complications and side effects; Denmark; Diagnosis; Dilatation, Pathologic; Disease Progression; Echocardiography; Female; Heart Defects, Congenital - complications; Heart Rate; Humans; Image Interpretation, Computer-Assisted; Imaging, Three-Dimensional; Linear Models; Magnetic Resonance Imaging; Middle Aged; Observer Variation; Predictive Value of Tests; Prospective Studies; Reproducibility of Results; Risk Assessment; Risk Factors; Time Factors; Turner syndrome; Turner Syndrome - complications; Young Adult
• ISSN: 1097-6647; 1532-429X; 1532-429X
• DOI: 10.1186/1532-429x-13-24

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. 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. 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. A general aortopathy is present in TS with enlargement of the ascending aorta, which is accelerated in the presence of a bicuspid aortic valve.