INTRODUCTION: The bicuspid aortic valve (BAV) is the most common congenital cardiac malformation. However there is a lack of studies relating the effect of high performance training and the size of the aortic root in elite athletes with BAV. PURPOSE: Evaluate the impact of BAV on aortic root remodeling in elite male athletes. METHODS: A retrospective cross-sectional study including 28 Spanish elite male athletes with BAV diagnosed by transthoracic echocardiography (age 23.2 ± 5.6 years, BSA 1.96 ± 0.16 m2, 8.7 ± 4 years/training and 17.2 ± 8.5 hours/day of training) were studied to assess end-diastolic aortic diameters in the parasternal long axis at: aortic annulus (AA), sinus of Valsalva (SV), sinotubular junction (SJ) and the maximum diameter of the proximal ascending aorta (AsPx); using the inner edge-to-inner edge (I-I) convention. The values were compared with a control group composed of 2041 healthy Spanish elite male athletes with normal tricuspid aortic valve (TAV) (age 24.1 ± 5.8 years, BSA 1.9 ± 0.2 m2, 9.6 ± 5.1 years/training and 19.2 ± 8.7 hours/day of training). Also the control group was used to establish the physiological limits of aortic remodeling associated to exercise training in elite athletes. RESULTS: BAV was associated with increased aortic dimension in comparison to the TAV at the level of AA (28.6 ± 4.8 mm vs 25.5 ± 3.3, p=0.001), SV (35.6 ± 5.7 vs 31.6 ± 3.6 mm, p=0.0001), SJ (29.7 ± 5.2 vs 26.4 ± 3.4 mm, p=0.0001) and AsPx (31.9 ± 6.9 vs 26.9 ± 3.6 mm, p=0.0001). Indexed aortic diameter was also increased in BAV at the level of AA (14.6 ± 2.5 vs 13.1 ± 1.7 mm/m2, p=0.002), SV (18.3 ± 3.2 vs 16.3 ± 1.9 mm/m2, p=0.001), SJ (15.2 ± 2.9 vs 13.6 ± 1.8 mm/m2, p=0.003) and AsPx (16.4 ± 3.8 vs 13.9 ± 1.9 mm/m2, p=0.0001). The dimensions of the aortic root in male elite athletes with BAV exceeding the upper normal range limit based on control group measurements were observed in 11 athletes at AA (39.3%), 11 at SV (39.3%), 8 at SJ (28.6%) and 8 at AsPx (28.6%). From this cohort, 7 elite male athletes with BAV (25%) had an aortic diameter greater than 40 mm at any level of measurement. CONCLUSIONS: Diameters of the aortic root in elite male athletes with BAV are greater than athletes with TAV at all levels of measurements. Almost two fifths of the athletes with BAV are over the physiological limit of the aortic root size.