Martín María, Lorca Rebeca, Rozado José, Alvarez-Cabo Rubén, Calvo Juan, Pascual Isaac, Cigarrán Helena, Rodríguez Isabel, Morís César
Cardiology Department, Instituto Reina Sofía de Investigación Nefrológica, REDinREN from ISCIII. Hospital Universitario Central de Asturias, Universidad de Oviedo, Oviedo, Spain.
Cardiac Surgery Department, Instituto Reina Sofía de Investigación Nefrológica, REDinREN from ISCIII. Hospital Universitario Central de Asturias, Universidad de Oviedo, Oviedo, Spain.
J Thorac Dis. 2017 May;9(Suppl 6):S454-S464. doi: 10.21037/jtd.2017.05.11.
Bicuspid aortic valve (BAV) or bicuspid aortopathy is the most common congenital heart disease. It can be clinically silent and it is often identified as an incidental finding in otherwise healthy, asymptomatic patients. However, it can be dysfunctioning at birth, even requiring neonatal intervention, or, in time, lead to aortic stenosis, aortic insufficiency, and endocarditis, and also be associated with aortic aneurysm and aortic dissection. Given its prevalence and significant complications, it is estimated that BAV is responsible for more deaths and morbidity than the combined effects of all the other congenital heart defects. Pathology of BAV is still not well known and many questions are unresolved. In this manuscript we review some aspects on bicuspid aortopathy, a heterogeneous and frequent disease in which like some authors have previously described, complex gene environment are present. Further investigations and, what is more, multidisciplinary teams are needed to improve our knowledge on this really fascinating disease.
二叶式主动脉瓣（BAV）或二叶式主动脉病变是最常见的先天性心脏病。它在临床上可能没有症状，常被发现于健康、无症状的患者，作为偶然发现。然而，它在出生时就可能功能异常，甚至需要新生儿期干预，或者随着时间发展，导致主动脉狭窄、主动脉瓣关闭不全和心内膜炎，还可能与主动脉瘤和主动脉夹层有关。鉴于其患病率和严重并发症，据估计，BAV导致的死亡和发病比所有其他先天性心脏缺陷的综合影响还要多。BAV的病理学仍不太清楚，许多问题尚未解决。在本手稿中，我们回顾了二叶式主动脉病变的一些方面，这是一种异质性且常见的疾病，正如一些作者之前所描述的，其中存在复杂的基因环境。需要进一步的研究，更重要的是，需要多学科团队来增进我们对这种真正迷人疾病的了解。
