Benini Annachiara, Benfari Giovanni, Pilati Mara, Luciani Giovanni Battista, Ribichini Flavio Luciano, Prioli Maria Antonia
Department of Medicine, Division of Cardiology, University of Verona, Verona, Italy.
Department of Surgery, Division of Cardiac Surgery, University of Verona, Verona, Italy.
J Cardiovasc Echogr. 2021 Jan-Mar;31(1):29-34. doi: 10.4103/jcecho.jcecho_115_20. Epub 2021 May 21.
Bicuspid aortic valve (BAV) is the most common congenital heart defect, with considerable risk of morbidity and mortality. The purpose of the study was to analyze clinical and echocardiographic presentation of BAV in a large-volume tertiary Italian center and to test their interaction with full age span, sex, and first diagnosis versus second referral.
Consecutive patients of all ages diagnosed with BAV at our center from January 1988 to December 2012 were retrospectively included. Exclusion criteria were as follows: associated complex congenital cardiac disease, systemic syndrome, and previous cardiac surgery.
Eligible patients were 790, divided by age quartiles. Seventy-two percent of patients had any grade BAV dysfunction. Aortic valve stenosis was more frequent in the first (24%) and fourth (24%) quartiles. This corresponds to a double-peak stenosis severity curve, being more severe at a very young age and in the elderly. Aortic valve regurgitation was more prevalent in each quartile than stenosis, with a prevalence of 72% in the second quartile and 77% in the third quartile. This corresponds to a single-peak regurgitation severity curve, being more severe in the fourth and fifth decades of life. Patients with previously diagnosed BAV had more significant valve dysfunction in comparison to patients with first diagnosis of BAV, either stenosis (15% vs. 21%, = 0.024) or regurgitation (58% vs. 68%, = 0.006).
The dominant BAV dysfunction in this large Northern Italian community is regurgitation, with higher severity of disease in the fourth and fifth decades of life.
二叶式主动脉瓣（BAV）是最常见的先天性心脏缺陷，具有较高的发病和死亡风险。本研究的目的是分析意大利一家大型三级医疗中心中BAV的临床和超声心动图表现，并检验其与全年龄范围、性别以及首次诊断与再次转诊之间的相互作用。
回顾性纳入了1988年1月至2012年12月在我们中心诊断为BAV的所有年龄段的连续患者。排除标准如下：合并复杂先天性心脏病、全身性综合征以及既往心脏手术史。
符合条件的患者有790例，按年龄四分位数分组。72%的患者存在任何程度的BAV功能障碍。主动脉瓣狭窄在第一（24%）和第四（24%）四分位数中更为常见。这对应于一条双峰狭窄严重程度曲线，在非常年轻和老年时更为严重。主动脉瓣反流在每个四分位数中比狭窄更为普遍，在第二四分位数中的患病率为72%，在第三四分位数中的患病率为77%。这对应于一条单峰反流严重程度曲线，在生命的第四和第五个十年中更为严重。与首次诊断为BAV的患者相比，先前诊断为BAV的患者瓣膜功能障碍更为明显，无论是狭窄（15%对21%，P = 0.024）还是反流（58%对68%，P = 0.006）。
在这个意大利北部的大型社区中，主要的BAV功能障碍是反流，在生命的第四和第五个十年中疾病严重程度更高。
