Bicuspid aortic valve

Dear Dr. Roach: I was diagnosed with a bicuspid aortic valve at age 54 (I’m now 62). I see a general cardiologist annually for an echocardiogram and an EKG, and have had one transesophageal echocardiogram. However, he has never ordered a CT or MRI to thoroughly check my aorta. I’ve read that BAV patients may have a connective tissue disorder, resulting in aortic problems (aneurysm, dissection).

My ascending aorta is mildly dilated, at 4 cm. Knowing that the size can change rapidly and without warning is a concern. Do you think I should insist on a CT or MRI? Other suggestions? — D.A.M.

Answer: The aortic valve, which separates the left ventricle from the aorta (the largest artery in the body), is supposed to have three leaflets. About 1 percent of people have only two leaflets, which is called a “bicuspid aortic valve.” Some of the people with bicuspid aortic valves also have risk for enlargement (dilation) of the aorta. You are absolutely right that this dilation can progress much more quickly than in people with three leaflets in their aortic valves.

An echocardiogram is recommended for young people with BAV every one or two years. For people whose aorta dilates to above 4 (right where you are now), the evaluation may need to be more frequent if the aorta is enlarging rapidly (in some people, it can dilate up to 0.9 mm per year).

I can’t answer the question about echocardiogram versus CT versus MRI, as the ideal imaging study varies from person to person. In some people, the regular echocardiogram gives good visualization; however, some people need advanced imaging, and I would generally prefer MRI, as it has no radiation and doesn’t usually need intravenous contrast for measuring the aorta.

You also should talk to your doctor about screening family members.