Abstract 10685: Bicuspid and ‘Partially Fused’ Aortic Valves are Common and Associated with Aortic Dilation Across Age Groups in Turner Syndrome
Background Women with Turner syndrome (TS) commonly have congenital aortic valve disease contributing to premature cardiovascular morbidity.
Methods The aortic valve and thoracic aorta were characterized by cardiovascular magnetic resonance imaging in 207 subjects in an IRB-approved natural history study of TS. Echo measured the peak velocity across the aortic valve, and the degree of aortic regurgitation.
Results Four distinct valve morphologies were identified: tricuspid aortic valve (TAV) (n=132), bicuspid aortic valve BAV (n=47), partial fusion (PF)(n=25) and unicuspid aortic valve (UAV) (n=3) (Figure 1). Aortic valve classification was completed by two reviewers, kappa=0.82. Right-left coronary cusp (91.9%) fusion predominated over right-non coronary cusp (8.1%) fusion. No significant differences in age, height, weight or BSA were found among the four groups. PF, BAV and UAV were significantly associated with mild aortic regurgitation and elevated peak velocities across the aortic valve. There was a significant trend towards larger ascending aorta diameters (AAD) from TAV to UAV, regardless of age and BSA (Figure 2).The mean AAD for TAV, PF, BAV and UAV were (mean +/- SD, p = 0.004) 24.8 +/- 4.6 mm, 27.2 +/- 4.3 mm, 29.4 +/- 5.9 mm and 30 +/- 4.3 mm respectively.
Conclusion Thirty-six percent of TS patients have aortic valve disease. Consistent with the hypothesis that development of the ascending aorta is abnormal from the aortic root to the ductus arteriosus in TS, aortic valve abnormalities were common and were associated with aortic root dilation. Figure 1. Gradient echo and phase contrast images of A) TAV, B) PF, C) BAV, D) UAV Figure 2. Aortic diameter by valve type
- © 2012 by American Heart Association, Inc.