Ortiz José T, Shin David D, Rajamannan Nalini M
Division of Cardiology, Northwestern University Feinberg School of Medicine, 303 E. Chicago Avenue, Tarry 12-717, Chicago IL 60611, USA.
Curr Treat Options Cardiovasc Med. 2006 Dec;8(6):461-7. doi: 10.1007/s11936-006-0034-6.
Bicuspid aortic valve (BAV) disease is a common congenital heart valve abnormality accounting for a large number of valve replacements in the United States. Although still incompletely understood, the natural history of BAV disease is severe aortic stenosis and associated ascending aortic dilatation. In addition to the increased risk of endocarditis, aortic dissection and severe aortic valve dysfunction are responsible for most fatal complications. Thus, early and precise recognition of this condition is mandatory. The new American College of Cardiology/American Heart Association recommendations highlight the role of MRI and CT as complimentary tools to echocardiography for the diagnosis and surveillance of the morphology of the aortic valve and ascending aorta. Moreover, better understanding of the cellular mechanisms, including inflammation, bone formation, atherosclerotic-like processes, and aortic wall abnormalities, as well as the heritability and genetic predisposition for the disease, will define the potential for targeted medical therapies in the future. Currently, the treatment of this condition is primarily surgical. Although combined valve and ascending aorta replacement has been the most common surgical approach in the past, the increased cumulative risk of thrombotic and embolic events among these young patients has led to more conservative approaches. Several valve-sparing approaches with comparable mid-term results compared with the classic procedures have recently been reported. However, longer follow-up studies will be helpful to better define the advantages of these new surgical options. After a quick overview of the natural history of the BAV, this article provides an updated approximation of the current knowledge of the pathophysiology as well as the recommendations for the management and treatment of this disease.
二叶式主动脉瓣（BAV）疾病是一种常见的先天性心脏瓣膜异常，在美国导致大量瓣膜置换手术。尽管对其仍未完全了解，但BAV疾病的自然病程是严重的主动脉瓣狭窄及相关升主动脉扩张。除了心内膜炎风险增加外，主动脉夹层和严重的主动脉瓣功能障碍是大多数致命并发症的原因。因此，早期准确识别这种疾病至关重要。美国心脏病学会/美国心脏协会的新指南强调了MRI和CT作为超声心动图的补充工具在诊断和监测主动脉瓣及升主动脉形态方面的作用。此外，更好地了解细胞机制，包括炎症、骨形成、动脉粥样硬化样过程和主动脉壁异常，以及该疾病的遗传度和遗传易感性，将为未来靶向药物治疗奠定基础。目前，这种疾病的治疗主要是手术治疗。尽管过去联合瓣膜和升主动脉置换一直是最常见的手术方法，但这些年轻患者中血栓形成和栓塞事件的累积风险增加，促使采用更保守的方法。最近有报道称，几种保留瓣膜的方法与传统手术相比，中期结果相当。然而，更长时间的随访研究将有助于更好地确定这些新手术选择的优势。在简要概述BAV的自然病程后，本文提供了当前病理生理学知识的最新概况以及该疾病管理和治疗的建议。
