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Background Although the incidence of aortic dissection is higher in patients with bicuspid aortic valve (BAV) compared to tricuspid aortic valve (TAV), risk stratification remains unclear. Guidelines focus on ascending aorta diameters, regardless of the location, and do not take into account the morphology of the aorta. Aortic tortuosity (AT) is emerging as a novel biomarker associated with more severe aortopathy in patients with Marfan syndrome. AT has not been accuretely assessed in BAV. Our aim is to describe the relationship between AT and ascending aortic phenotype in patients with BAV. Methods 83 patients (43±16 years, 19 women) diagnosed with BAV and without significant aortic valve disease nor prior aortic intervention were included. CT scans were retrospectively analysed with measurements of aortic diameters and aortic tortuosity. For 61 patients with abdominal images available, descending and total aortic length and tortuosity were measured. Results In our cohort, 62 (75%) patients presented a typical BAV. Pathological aorta (Root and/or tubular Z-score >2) was found in 80 patients (96%) and 67 (81%) presented a tubular dilatation. The aortic phenotype, the maximal aortic diameters and aortic tortuosity index were similar in typical and atypical BAV. Total aortic tortuosity index was correlated to Z-score tubular diameter (r=0.31; p=0,014) but not with Z-score Valsalva diameter (p=0,55). In patients with tubular dilatation (Z score >2), total aortic tortuosity index was higher than in patient without tubular dilatation (2.01 vs 1.85; p=0,015). Conclusion Total aortic tortuosity is associated with tubular dilatation but not with root dilatation in BAV patients suggesting that tubular phenotype may be at higher risk of complication in BAV. Further studies evaluating the association between aortic tortuosity and clinical outcomes in BAV are needed. Funding Acknowledgement Type of funding sources: None.

Introduction Sickle cell disease is the most frequent genetic hemoglobinopathy worldwide and early childhood mortality has dramatically decreased in high-income countries. However, in the aging sickle cell disease population, the morbidity related to chronic organ damage, especially kidney and heart, has become a major concern. The disease is well characterized in homozygous SS patients, but the complications associated with Haemoglobin SC disease (HbSC) are mostly unknown. Dyspnea is frequent in SS patients and associated with poor quality of life, this symptom was not investigated in HbSC patients. Our objective is to investigate the prevalence and the factors associated with dyspnea in HbSC patients. Method A total of 221 HbSC patients (mean age 35 years, 47% men) were prospectively investigated. Clinical and biological data were collected, and 109 (49%) patients underwent an echocardiography. Results A dyspnea was observed in 28 (13%) patients. Compared to the rest of the cohort, these patients were older and had a lower hemoglobin level (P Fig. 1 ). Conclusion Dyspnea is relatively uncommon in adult HbSC patients and related to age and hemoglobin level. Cardiac involvement seems not to be a major factor associated to dyspnea in HbSC patients.