Endocrine Abstracts

Introduction
Cardiovascular complications are the main cause of increased mortality in Turner Syndrome (TS). We have previously described a high prevalence of occult aortic dysmorphology in women with TS. The aim of this study was to introduce a classification system for the variety of appearances seen by magnetic resonance imaging (MRI).
Method
98 women (median age 29.0, range 18.1-64.2 years) underwent MRI scanning of the aorta. MRI appearances were correlated with echocardiography findings (available in 87 women) and clinical parameters, and categorised as gross aortic arch morphology, degree of coarctation and flow disturbance (FD) at the classical coarctation site, and aortic root dilatation (ARD).
Results
Completely normal aortic morphology without coarctation or ARD was found in only 30.6% of women. 42.9% had a range of abnormalities at the coarctation site, including non-significant coarctation without FD (5.1%), non-significant coarctation with FD (14.3%), significant coarcation with FD (8.2%), and previously repaired coarctation (7.1%).
Other morphological aortic arch abnormalities noted were an elongated ascending limb with clinically non-significant pseudocoarctation (5.1%), elongated upper limb (2.0%), elongated descending limb (9.2%) and square aortic arch (14.3%). A morphological variant with tortuosity was found in 15.3%.
35.7% had ARD as defined by a ratio >/=1.5 of ascending to descending aortic diameter, whilst 20.5% had ARD by echocardiography criteria. Bicuspid aortic valve on echocardiography was positively correlated with coarctation, ARD and left ventricular hypertrophy.
Karyotype monosomy 45,X was associated with overall abnormality including all aspects of aortic morphology (chisquare p=0.040). Blood pressure and age correlated with ascending aortic diameter by MRI (p<0.01), and age correlated with severity of coarctation in these asymptomatic women (p=0.015).
Conclusion
These findings show that there is considerable occult aortic pathology in asymptomatic adults with TS, both significant and non-significant. The future clinical significance of these findings should become apparent with continuing follow-up.