Department of Pediatrics, Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Canada (S.B., F.D.).
Department of General Surgery, Maisonneuve-Rosemont Hospital, Montreal, Canada (L.M.-D.).
Circ Cardiovasc Imaging. 2020 Mar;13(3):e009675. doi: 10.1161/CIRCIMAGING.119.009675. Epub 2020 Mar 17.
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.
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 scores) were modeled in relation to age and potential predictive variables in a linear mixed model. The primary outcome was the rate of dilatation.
We included 761 subjects (3134 echocardiograms) in final analyses. The mean ascending aorta score progression rate for BAV patient with a normally functioning aortic valve was estimated at 0.05 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.
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.
二叶式主动脉瓣（BAV）患者发生主动脉瓣功能障碍和进行性升主动脉扩张的风险较高，这可能导致主动脉夹层。时至今日，在儿科随访期间识别有发生严重主动脉扩张风险的儿童仍然具有挑战性，因为大多数研究都局限于成年患者。本研究的总体目标是确定二叶式主动脉瓣患儿主动脉扩张的危险因素。
我们提取了 1999 年至 2016 年期间在圣贾斯汀大学中心医院接受随访的所有 0 至 20 岁的二叶式主动脉瓣患者的临床和超声心动图数据。我们排除了伴有伴发心脏缺陷和影响近端主动脉尺寸的患者。采用线性混合模型，根据年龄和潜在预测变量，对近端主动脉直径（表示为 Z 评分）与年龄的关系进行建模。主要结局为扩张率。
我们最终纳入了 761 名患者（3134 次超声心动图）进行分析。主动脉瓣功能正常的二叶式主动脉瓣患者升主动脉 Z 评分的平均进展率估计为每年 0.05 个 Z 评分单位。预测扩张率增加的最强因素是严重主动脉瓣狭窄、中度和重度主动脉瓣反流以及未经矫正的主动脉缩窄。主动脉瓣叶融合模式和性别与进展率无关。
主动脉瓣功能正常的儿童升主动脉扩张速度非常缓慢。主动脉瓣功能障碍超过轻度的患者升主动脉扩张率显著增加，但与二叶式主动脉瓣叶融合类型无关。
