To investigate the association between bicuspid aortic valve (BAV) morphologic findings (raphe vs nonraphe), the degree of valve dysfunction and prognosis.

Clinical and echocardiographic data in 317 BAV patients were analyzed retrospectively. According to the Sievers classification, the morphologic BAV findings were categorized into no raphe (type 0), one raphe (type 1) and two raphes (type 2). The patients with type 1 were further divided into three subtypes, including R-L subtype (fusion of the left and right coronary cusps), R-N subtype (fusion of the right and noncoronary cusps) and L-N subtype (fusion of the left and noncoronary cusps).

Of the 317 patients, there were 83 (26.2%) of type 0, 232 (73.2%) of type 1 and 2 (0.6%) of type 2.Among the 232 patients of type 1, there were 126 (54.3%) of R-L subtype, 88 (37.9%) of R-N subtype and 18 (7.8%) of L-N subtype. BAV with raphe had a significantly higher prevalence of aortic valve calcification [120 (51.3%) vs 19 (22.9%), P<0.001], with significantly higher frequencies of aortic stenosis [164 (70.1%) vs 6 (7.2%), P<0.001], aortic regurgitation [168 (71.8%) vs 40 (48.2%), P=0.001], increased left ventricular mass[(253.4±113.7)g vs (176.4±69.3)g, P<0.001] and left heart failure [34 (14.5%) vs 3 (3.6%), P=0.009]. Furthermore, the dilation of aortic root and ascending aorta in BAV patients with raphe were significantly higher than those without raphe (P<0.01), however, ascending aortic aneurysm rates were not significant between BAV with and without raphe[23(9.8%) vs 4(4.8%), P=0.251]. The patients in R-N subtype had a significantly higher proportion of aortic valve calcification than those in R-L and L-N subtype [55 (62.5%) vs 57 (45.2%) vs 6 (33.3%), P=0.01], with a significantly higher frequency of severe aortic stenosis [50 (56.8%) vs 21 (16.7%) vs 3 (16.7%), P<0.001]. However, there was no significant difference among different subtypes in other complications(P>0.05).

The presence of raphe is associated with a higher frequency of significant aortic valve calcification, aortic valve dysfunction, and increases left ventricular mass and left heart failure. The R-N type is also associated with aortic valve calcification and severe aortic stenosis.

Echocardiography; Aortic valve stenosis; Aortic valve insufficiency; Bicuspid aortic valve; Complication