Centre de Référence pour le Syndrome de Marfan et Apparentés, Hôpital Bichat, AP-HP, Paris, France; Service de Cardiologie, Hôpital Bichat, AP-HP, Paris, France.
Am J Cardiol. 2010 Mar 15;105(6):888-94. doi: 10.1016/j.amjcard.2009.11.040.
The evaluation of aortic root dilation is of major importance for the diagnosis and follow-up of patients with diverse diseases, including the Marfan syndrome. However, we noted that the available nomograms suggested a lower aortic root dilation rate in adults (75%) than in children (90%), when the opposite would have been expected. To establish new nomograms, we selected a population of 353 normal children. We took transthoracic echocardiographic measurements of the aortic root diameters at the level of the aortic annulus, sinuses of Valsalva, sinotubular junction, and ascending aorta according to the American Society of Echocardiography recommendations. All diameters correlated well with the height, weight, body surface area, and age (r = 0.75 to 0.84, p <0.0001). Covariance analysis adjusting for body surface area showed slightly larger diameters at the level of the sinuses of Valsalva in male children than in female children (+1 mm, p = 0.0002). Equations and derived nomograms were developed, giving the upper limit of normal (allowing simple recognition of aortic dilation) and the Z score (allowing fine quantification of dilation and differentiation of normal growth from pathologic dilation) for all 4 aortic root diameters (ie, aortic annulus, sinuses of Valsalva, sinotubular junction, and proximal ascending aorta) according to body surface area and gender. We applied the nomograms to 282 children with confirmed Marfan syndrome, of whom 65.2% presented with dilation of the sinuses of Valsalva. In conclusion, we propose equations to calculate the upper limit of normal and Z-score for aortic root diameters measured by 2-dimensional echocardiography, which should be useful tools for the diagnosis and follow-up of aortic root aneurysms in children.
主动脉根部扩张的评估对各种疾病患者的诊断和随访具有重要意义，包括马凡综合征。然而，我们注意到，现有的列线图提示成年人（75%）主动脉根部扩张率低于儿童（90%），这与预期的情况相反。为了建立新的列线图，我们选择了 353 名正常儿童的人群。根据美国超声心动图学会的建议，我们对主动脉瓣环水平、主动脉窦、窦管交界和升主动脉的主动脉根部直径进行经胸超声心动图测量。所有直径均与身高、体重、体表面积和年龄密切相关（r = 0.75 至 0.84，p <0.0001）。调整体表面积的协方差分析显示，男性儿童主动脉窦水平的直径略大于女性儿童（+1mm，p = 0.0002）。我们开发了方程和衍生的列线图，为所有 4 个主动脉根部直径（即主动脉瓣环、主动脉窦、窦管交界和近端升主动脉）提供了正常上限（允许简单识别主动脉扩张）和 Z 评分（允许精细量化扩张，并区分正常生长和病理性扩张），这些都是根据体表面积和性别得出的。我们将这些列线图应用于 282 名确诊为马凡综合征的儿童，其中 65.2%的患儿存在主动脉窦扩张。总之，我们提出了用于计算二维超声心动图测量的主动脉根部直径的正常上限和 Z 评分的方程，这些方程应该是儿童主动脉根部瘤诊断和随访的有用工具。
