BACKGROUND Bicuspid aortic valve (BAV) is the most prevalent congenital heart defect affecting 1% to 2% of the population. It is associated with ascending aorta dilatation. Valve morphology, aortic stenosis (AS), and aortic insufficiency (AI) have been proposed as potential risk factors; however, evaluating their role is difficult, as these factors are inherently related. The aim of this study was to determine whether BAV morphology and dysfunction are independent determinants for ascending aorta dilatation in pediatric patients. METHODS A multicenter, retrospective, cross-sectional study of pediatric BAV patients followed since 2004 was performed. Imaging data were assessed for BAV morphology, severity of AS and AI, history of coarctation, and aortic dimensions. Associations were determined using multivariable regression analysis. A subset of patients undergoing aortic interventions (balloon dilation or Ross) were assessed longitudinally. RESULTS Data were obtained from 2122 patients (68% male; median age 10.2 years). Fifty percent of patients had ascending aorta dilatation. Right and noncoronary cusp fusion, increasing AS and AI, and older age were independently associated with ascending aorta dilatation. A history of coarctation was associated with less ascending aorta dilatation. In patients with neither AS nor AI, 37% had ascending aorta dilatation (4% severe). No complications related to aortic dilatation occurred in this cohort. Aortic Z scores were determined, and a Z-score calculator was created for this population. CONCLUSIONS In this large pediatric cohort of patients with BAV, valve morphology, AS, and AI are independently associated with ascending aorta dilatation, suggesting that hemodynamic factors influence aortopathy. However, even in BAVs with no AS or AI, there is significant ascending aorta dilatation independent of valve morphology. Interventions that led to changes in degree of AI and AS did not seem to influence change in aortic dimensions. The current BAV cohort can be used as a reference group for expected changes in aortic dimensions during childhood.

中文翻译：

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儿童二尖瓣主动脉瓣相关性主动脉病变的预测因素：MIBAVA协会的报告。

背景技术二尖瓣主动脉瓣（BAV）是最普遍的先天性心脏缺陷，影响了1％至2％的人口。它与升主动脉扩张有关。瓣膜形态，主动脉瓣狭窄（AS）和主动脉瓣关闭不全（AI）被认为是潜在的危险因素。但是，由于这些因素具有内在的联系，因此很难评估它们的作用。这项研究的目的是确定BAV形态和功能障碍是否是小儿患者升主动脉扩张的独立决定因素。方法自2004年以来，对儿童BAV患者进行了多中心，回顾性横断面研究。评估成像数据的BAV形态，AS和AI的严重程度，缩窄的历史以及主动脉的大小。使用多变量回归分析确定关联。纵向评估接受主动脉干预（气球扩张或罗斯）的部分患者。结果数据来自2122例患者（68％男性；中位年龄10.2岁）。50％的患者患有升主动脉扩张。右冠状动脉和非冠状动脉的融合，AS和AI的增加以及年龄的增长与升主动脉扩张独立相关。缩窄史与升主动脉扩张较少有关。在既没有AS也没有AI的患者中，有37％的患者升主动脉扩张（严重者为4％）。该队列未发生与主动脉扩张相关的并发症。确定主动脉Z评分，并为此人群创建Z分数计算器。结论在这个有BAV，瓣膜形态，AS，AI和AI与升主动脉扩张独立相关，提示血液动力学因素影响主动脉病变。但是，即使在没有AS或AI的BAV中，也有明显的升主动脉扩张，而与瓣膜形态无关。导致AI和AS程度改变的干预措施似乎并未影响主动脉尺寸的改变。当前的BAV队列可以用作儿童期主动脉尺寸预期变化的参考组。