Aortic Valve Dysfunction and Aortopathy Based on the Presence of Raphe in Patients with Bicuspid Aortic Valve Disease

(1) Background: To identify the association between the presence or absence of a raphe and aortic valve dysfunction, as well as the presence of aortopathy in patients with a bicuspid aortic valve (BAV); (2) Methods: This retrospective study enrolled 312 participants (mean (SD) age, 52.7 (14.3) years; 227 men (72.8%)) with BAV. The BAVs were divided into those with the presence (raphe+) or absence (raphe−) of a raphe. Valvular function was classified as normal, aortic regurgitation (AR), or aortic stenosis (AS) using TTE. The pattern of BAV aortopathy was determined by the presence of dilatation at the sinus of Valsalva and the middle ascending aorta using CCT; (3) Results: BAVs with raphe+ had a higher prevalence of AR (148 (79.5%) vs. 48 (37.8%), p < 0.001), but a lower prevalence of AS (90 (48.6%) vs. 99 (78.0%), p < 0.001) compared with those with raphe−. The types of BAV aortopathy were significantly different (p = 0.021) according to those with BAV–raphe+ and BAV–raphe−; (4) Conclusions: The presence of a raphe was significantly associated with a higher prevalence of AR, but a lower prevalence of AS and combined dilatation of the aortic root and middle ascending aorta. The presence of a raphe was an independent determinant of AR.