摘要目的 探讨二叶主动脉瓣(BAV )的不同解剖形态对瓣膜结构、功能及转归的影响.方法 回顾分析317例BAV患者,根据Sievers分型标准将BAV分为无嵴型(type 0)和有嵴型,根据嵴的数目将有嵴型分为1个嵴(type 1)和2个嵴(type 2) ,再按照瓣膜融合情况将type 1分为右冠瓣‐左冠瓣融合(R‐L ) 、右冠瓣‐无冠瓣融合(R‐N )及左冠瓣‐无冠瓣融合(L‐N )三个亚型,并比较不同类型BAV患者的临床及超声心动图特征.结果 ①317例BAV患者中 type 0者83例(26 .2%) , type 1者232例(73 .2%) , type 2者2例(0 .6%) ,其中type 1中R‐L亚型126例(54 .3%) , R‐N亚型88例(37 .9%) , L‐N亚型 18 例(7 .8%) .② 有嵴型 BAV 患者较无嵴型更易发生主动脉瓣钙化[120 (51 .3%)对 19 (22 .9%) ,P ＜0 .001 ] ,主动脉瓣狭窄发生率更高[164 (70 .1%)对6 (7 .2%) , P ＜0 .001] ,主动脉瓣反流发生率更高[168 (71 .8%)对40 (48 .2%) ,P ＝0 .001] ,左室心肌质量明显增加[ (253 .4 ± 113 .7) g 对(176 .4 ± 69 .3) g ,P ＜0 .001] ,且并发左心衰竭者比例更高[34 (14 .5%)对 3 (3 .6%) ,P ＝0 .009] ;有嵴型BAV患者主动脉根部及升主动脉均稍宽( P ＜0 .01) ,但达到升主动脉瘤程度者两组间差异并无统计学意义[23 (9 .8%)对4 (4 .8%) ,P ＝0 .251] .③R‐N亚型主动脉瓣钙化发生率比R‐L亚型和L‐N亚型稍高[55 (62 .5%)对57 (45 .2%)对6 (33 .3%) ,均 P ＝0 .01] ,且并发主动脉瓣狭窄更多[79 (89 .8%) , 70 (55 .6%) , 13 (72 .2%) ,均 P ＜0 .001] ,尤其主动脉瓣重度狭窄发生率更高[50 (56 .8%)对21 (16 .7%)对 3 (16 .7%) ,均 P ＜0 .001] ,而其他并发症发生率在各亚型间差异无统计学意义( P ＞0 .05) .结论 有嵴型BAV患者更易发生主动脉瓣钙化及功能障碍、左室心肌质量增大,且并发左心衰竭比例高,R‐N亚型主动脉瓣钙化及重度狭窄发生率更高.

abstractsObjective To investigate the association between bicuspid aortic valve ( BAV ) morphologic findings ( raphe vs nonraphe) ,the degree of valve dysfunction and prognosis . Methods 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 ) . T he 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 ) . Results 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] . T he 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).Conclusions T he 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 .T he R‐N type is also associated with aortic valve calcification and severe aortic stenosis .