Background:Patients with bicuspid aortic valve (BAV) have a higher risk of developing aortic valve dysfunction and progressive proximal aorta dilatation, which can lead to aortic dissection. To this day, identification of children at risk of developing severe aortic dilatation during their pediatric follow-up is still challenging because most studies were restricted to adult subjects. The overarching goal of this study was to identify risk factors of aortic dilatation in children with BAV.Methods:We extracted clinical and echocardiographic data of all BAV subjects aged 0 to 20 years followed at Centre Hospitalier Universitaire Sainte-Justine between 1999 and 2016. We excluded subjects with concomitant heart defects and conditions affecting proximal aorta dimensions. Proximal aorta diameters (expressed as Z scores) were modeled in relation to age and potential predictive variables in a linear mixed model. The primary outcome was the rate of dilatation.Results:We included 761 subjects (3134 echocardiograms) in final analyses. The mean ascending aorta Z score progression rate for BAV patient with a normally functioning aortic valve was estimated at 0.05 Z score unit per year. The strongest predictors of an increased dilatation rate were severe aortic stenosis, moderate and severe aortic regurgitation, and uncorrected coarctation of the aorta. Aortic valve leaflet fusion pattern and sex were not associated with progression rate.Conclusions:Children with a normally functioning BAV exhibited a very slow proximal aorta dilatation rate. Ascending aorta dilatation rate was significantly increased in patients with more than mild aortic valve dysfunction but was independent from BAV leaflet fusion type.

中文翻译：

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在二尖瓣主动脉瓣儿童大队列中近端主动脉扩张的长期危险因素。

背景：患有双尖瓣主动脉瓣（BAV）的患者发生主动脉瓣功能障碍和进行性主动脉近端扩张的风险较高，这可能导致主动脉夹层分离。迄今为止，由于大多数研究仅限于成年受试者，因此在儿科随访期间确定有发生严重主动脉扩张风险的儿童仍然具有挑战性。本研究的总体目标是确定BAV儿童主动脉扩张的危险因素。方法：我们提取了1999年至2016年在Saint-Justine大学医院进行的0至20岁所有BAV受试者的临床和超声心动图数据。排除伴有心脏缺损和影响近端主动脉尺寸的疾病的受试者。主动脉近端直径（表示为Z分数）在线性混合模型中与年龄和潜在的预测变量有关。结果：我们纳入了761例受试者（3134例超声心动图）。具有正常功能的主动脉瓣的BAV患者的平均升主动脉Z评分进展速度估计为0.05 Z每年的得分单位。扩张率增加的最强预测因素是严重的主动脉瓣狭窄，中度和严重的主动脉瓣关闭不全以及主动脉缩窄。结论：BAV正常的儿童的主动脉近端扩张速度很慢。轻度以上主动脉瓣功能不全的患者升主动脉扩张率显着增加，但与BAV小叶融合类型无关。