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Introduction: Bicuspid aortic valve (BAV) is an phenotypically heterogeneous disorder: - type I BAV involves right-left cusp fusion, prominent aortic dilatation and association with coarctation, while type II involves right-non coronary cusp fusion and is associated with early development of valve stenosis/regurgitation. As regards pathogenesis, Type II BAV has been linked to impairment of nitric oxide (NO) effect, but there is little information on type I. We therefore sought to determine whether development of valve stenosis or aortic dilatation in Type I BAV is correlated with markers of endothelial dysfunction, NO hyporesponsiveness, inflammatory activation or increased endothelial cell turnover.Methods: We evaluated 33 patients with BAV aged 20 to 60 years, utilizing peak aortic flow velocity (PAV); (excluding patients with significant aortic regurgitation), and diameter of the aortic sinus (ASD) respectively as indices of valve and aortic disease. These were correlated with: (i) flow-mediated dilatation (FMD), anti-aggregatory effects of the NO donor sodium nitroprusside (SNP), (iii) hsCRP and (iv) endothelial progenitor cell (CD34/CD133 positive cells) count [EPC].Results: Ageing was associated with gradual increases in ASD and PAV (p =0.08 and 0.012), respectively. Of the four comparators, only hsCRP elevation was a significant correlate of high PAV, (figure), while increases in ASD were associated with increased EPC count (figure). FMD values were within normal population ranges for 70% of patients.Conclusion: In type I BAV there is no strong association with either endothelial dysfunction or hyporesponsiveness to NO. Rather inflammatory activation and increased EPC release are correlates of development of valve and arch disease respectively.