Management of patients with bicuspid aortic valve disease.

Our approach to the management of the patient with a bicuspid aortic valve (BAV) takes several factors into consideration. First, is the dysfunction of the valve due to aortic stenosis (AS), aortic regurgitation (AR), or a combination of stenosis and regurgitation, and what is the severity? Next, is there aortic dilation in any of the regions (sinuses of Valsalva, sinotubular junction, tubular ascending aorta, or transverse arch) discussed in this article. In general, we follow patients with a BAV and moderate valve dysfunction (AS or AR) with yearly surveillance transthoracic echocardiography for left ventricular function, jet velocity, gradient, and valve area with AS, whereas left ventricular (LV) function and LV dimensions are monitored for patients with AR. In addition, yearly clinical evaluation for change in symptom status or functional capacity is critical. More recently, we have utilized NT-pro BNP levels to help assess patients, particularly those in whom the anatomic severity does not match the clinical symptoms (ie, the valve severity appears mild but the patient is complaining of symptoms or the valve severity seems significant but no symptoms are noted). All patients with a bicuspid valve should have evaluation of the aorta with a MRI or CT angiography at some point, as 50% of BAV patients have aortic root involvement. At our institution, cardiac MRI is preferred unless there is a contraindication, particularly in younger patients, given the cumulative radiation exposure from surveillance CT scans. Cardiac MRI also provides the added benefit of information regarding LV function, LV dimensions, and assessment of valve stenosis/regurgitation severity, thus obviating the need for echocardiographic data in those being followed with serial cardiac MRI. For those with no aortic dilatation, we tend to use only echocardiography for follow-up. For patients with mild aortic dilation, surveillance aortic imaging is usually performed every 3-5 years. However, for those with greater degrees of aortic dilation (aortic diameters >4.0 cm) or notable interval change in dimensions, then aortic imaging every year is conducted. For young adult patients with isolated aortic stenosis, balloon aortic valvuloplasty is often an effective and temporizing treatment option. In older patients with aortic stenosis or those with AR, aortic valve replacement, with or without a surgery on the aorta depending on whether concomitant dilation (aortic diameter >4.5 cm) of the aorta is present, is the preferred management strategy. In a few patients, surgery on the aortic alone may be indicated if the maximal diameter exceeds 5.0 cm.