Yuan Shi-Min, Jing Hua
Department of Cardiothoracic Surgery, Jinling Hospital, School of Clinical Medicine, Nanjing University, Jiangsu Province, People's Republic of China.
Sao Paulo Med J. 2010;128(5):296-301. doi: 10.1590/s1516-31802010000500010.
Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, affecting 1-2% of the population, with strong male predominance. Individuals may have a normally functioning BAV, and may be unaware of its presence and the potential risk of complications. However, they may easily develop aortic valve disorders: either stenotic or regurgitant, or both. Today, BAV is recognized as a syndrome incorporating aortic valve disorders and aortic wall abnormalities, including aortic dilation, dissection or rupture. Congenital or hereditary diseases such as ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, Turner's syndrome, Marfan's syndrome etc., may frequently be associated with BAV. Infective endocarditis and occasionally thrombus formation may develop during the lives of BAV patients. Elevated cholesterol or C-reactive protein may be seen in laboratory findings of these patients. Beta-blockers and statins are the possibilities for medical treatment, and aortic valve repair/replacement and ascending aorta replacement are indicated for patients with a severely diseased aortic valve and aorta. Rigorous follow-up throughout life is mandatory after BAV has been diagnosed. The aim of the present article was to describe the implications of BAV and its associated disorders, and to discuss diagnostic and treatment strategies.
二叶式主动脉瓣（BAV）是最常见的先天性心脏畸形，影响1%-2%的人口，男性占比极高。个体可能有功能正常的BAV，且可能未意识到其存在及并发症的潜在风险。然而，他们可能容易出现主动脉瓣疾病：狭窄或反流，或两者皆有。如今，BAV被认为是一种包含主动脉瓣疾病和主动脉壁异常（包括主动脉扩张、夹层或破裂）的综合征。先天性或遗传性疾病，如室间隔缺损、动脉导管未闭、主动脉缩窄、特纳综合征、马凡综合征等，常与BAV相关。BAV患者在其一生中可能发生感染性心内膜炎，偶尔还会形成血栓。这些患者的实验室检查结果可能会出现胆固醇或C反应蛋白升高。β受体阻滞剂和他汀类药物是药物治疗的选择，对于主动脉瓣和主动脉严重病变的患者，需进行主动脉瓣修复/置换及升主动脉置换。BAV确诊后，终身严格随访是必要的。本文旨在描述BAV及其相关疾病的影响，并讨论诊断和治疗策略。
