Approach to the patient with bicuspid aortic valve and ascending aorta aneurysm

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.