Cedars-Sinai Heart Institute - Questions about Bicuspid Aortic Disease

Questions about Bicuspid Aortic Disease

I have been told I have a bicuspid aortic valve. I also have an ascending aortic aneurysm. Did my valve cause this aneurysm?

Bicuspid aortic valves and ascending aortic aneurysms often occur in the same person. At one time it was thought that stress on the aortic wall from the abnormal blood flow through the bicuspid valve damaged the aorta, causing it to enlarge. However, in some people the bicuspid aortic valve has been replaced and an aortic aneurysm still developed years later, even though the aorta was no longer subject to the abnormal blood flow pattern. It has been found that the aortic tissue itself may be weak. This weakness is due to connective tissue degeneration in the wall of the aorta, making it prone to aneurysm formation.

My bicuspid aortic valve was replaced five years ago. With the defective valve gone, am I protected from getting an aortic aneurysm?

No, as mentioned in the answer to the question above, the aortic tissue may be abnormal. Your aorta should be monitored on an ongoing basis to detect any enlargement at the earliest stage possible. Once the aortic diameter reaches 4.5 cm, prophylactic surgery should be done. Please see Timing of Elective Surgery for further details.

I had coarctation of the aorta repaired as a child. I have recently been diagnosed with an ascending aortic aneurysm of 4.6 cm. My aortic valve is also bicuspid. Did the coarctation cause this problem I have now? Are there special considerations for someone like me regarding aortic surgery?

Coarctation (narrowing) of the aorta may be found along with bicuspid aortic valves. The underlying reason why they occur together in some people and not in others is not understood. However, what is clear is that there is a genetic condition that may include both bicuspid aortic valves and weakened aortic tissue. With the passage of time, the aortic tissue continues to weaken and degenerate until the walls of the aorta balloon out, which is called an aneurysm. Generally, with the presence of bicuspid aortic disease (BAD) and an ascending aortic aneurysm greater than 4.5 cm prophylactic, surgical replacement of the aorta is recommended.

I understand that those with bicuspid aortic valves may also be at risk of dissection or aneurysm in the ascending aorta. I have been told that my bicuspid aortic valve must be replaced. My aorta is slightly enlarged. Are there any tests to see if my aorta is healthy or not? Should my aorta be replaced at the same time, or should it just be monitored, hoping that it will not enlarge further?

Generally speaking, a slightly enlarged aorta in the presence of a bicuspid aortic valve (BAV) has a higher incidence of aneurysm formation and dissection. Unfortunately, there are no tests that can predict whether or not the aorta will continue to enlarge or be prone to dissection in the future. Because of this and the high mortality rate associated with acute ascending aortic dissection in those with bicuspid aortic valves, specialists in aortic surgery recommend that a slightly enlarged ascending aorta be replaced at the time of valve replacement.

My father and uncle both have bicuspid aortic valves and enlarged aortas. I have just been told that I have a bicuspid valve. If I have children some day, will they also have this condition?

It is known that bicuspid aortic disease is inherited. However, it is impossible to predict exactly who will be affected. In some families, each generation has one or more members affected. In other families, generations may be "skipped." It is possible that even if none of your children have this disease, others family members (such as your nieces, nephews or grandchildren) might be affected. Careful monitoring of all family members is required with echocardiography, even if their aortic valve appears normal.