摘要
尽管二叶式主动脉瓣(BAV)患者易患升主动脉(AA)扩张、狭窄和夹层,但对BAV患儿主动脉疾病的进展情况却鲜有记载。本研究的目的是确定BA V患儿AA直径的变化速率,并探讨主动脉扩张进展的危险因素。回顾性分析了276例年龄【19岁(平均8.5±5.3岁)孤立性BAV患儿的超声心动图资料。将主动脉测量结果用体表面积标化为z分数。在包括112例接受连续检查的患者的亚组中,计算主动脉扩张速率,并确定加速主动脉扩张的危险因素。最初时,33例(12%)患者有显著的AA扩张(z】4),70例(25%)为轻度异常(z在2～4之间)
Although patients with bicuspid aortic valves(BAVs) are predisposed to ascending aortic(AA) dilation, stenosis, and dissection, the development of aortic disease in children with BAVs is poorly described. The purposes of this study were to determine the rate of change of AA diameter in children with BAVs and to identify risk factors for the development of aortic dilation. The echocardiograms of 276 children aged< 19 years(mean 8.5± 5.3) with isolated BAVs were reviewed. Aortic measurements were normalized to z scores on the basis of body surface area. In a subset of 112 patients with serial examinations, aortic growth rates were calculated and risk factors for more rapid aortic growth determined. At presentation, 33 patients(12% ) demonstrated marked AA dilation(z >4), and 70(25% ) were moderately abnormal(z between 2 and 4). The mean± SD AA diameter increased more than expected, at a rate of 0.18± 0.30 z score per year(p< 0.0001). In 61 patients with normal AA diameters on initial study, 22(36% ) had abnormal diameters, with z scores >2, at follow-up. Univariate analysis demonstrated right-noncoronary commissural fusion(p< 0.02) and aortic valve gradient on initial examination(p< 0.02) as significant predictors of AA growth. In multivariate analysis, however, the significance of gradient and valve morphology was diminished(p=0.06 for both). In conclusion, the progression of AA diameter in patients with normal z scores on initial examination suggests that serial echocardiograms are required to screen for the development of significant aortic dilation.
