Re. Quadricuspid Aortic Valve
Re. Quadricuspid Aortic Valve
Dear Michelle,
I read your original post, and I am concerned that you mention chest pain. I want to encourage you to have an evaluation of your aorta.
Malformations of the aortic valve include unicuspid (one leaflet, looks like a donut); bicuspid (two leaflets, sometimes called a "fish mouth" valve); and quadricuspid (four leaflets).
All of these abnormal forms of the aortic valve may be associated with abnormal aortic tissue also. You need to have your aorta evaluated for enlargement/aneurysm - this could be the source of your pain. The entire aorta can be scanned with an MRI or CT (with contrast) and accurately measured in a center with expertise in the thoracic aorta. Also, has anyone counseled you about blood pressure?
Here is one reference from the literature regarding aneurysm with a quadricuspid aortic valve
http://www.ncbi.nlm.nih.gov/entrez/...uids=15514607&query_hl=15&itool=pubmed_docsum
The quadricuspid apparently is very rare. I can only tell you if this were in my own body, I would seek out expertise in a major center. Even then it will be difficult to find someone who has seen and treated very many of these. For example, here is a paper regarding replacing the quad aortic valve and techniques that may help avoid complete heart block. There is some risk with AVR in coming too close to an "electrical" node, and it can result in needing a pacemaker after surgery. This paper from Brigham and Women's describes special considerations when it is a quad aortic valve.
http://www.ncbi.nlm.nih.gov/entrez/...uids=16731182&query_hl=14&itool=pubmed_docsum
Many centers still are unaware of the full range of complexities with the quite common bicuspid aortic valve. The special considerations for a quadricuspid aortic valve, which is much more rare, will be even less understood. The best chance of getting state of the art knowledge and avoiding complications now and surprises later is to actively seek expertise. Reading this post, the plans for you as described are what is typically done for a "routine" valve assessment. Again, if it were myself, I would not be comfortable with that.
If nothing else, insist that your aorta be checked for an aneurysm. You want to avoid aortic dissection or rupture at all costs.
Best wishes,
Arlyss