There Are Still Not Enough Answers.....
There Are Still Not Enough Answers.....
Bicuspid aortic valves are emerging from being mentioned only briefly in cardiology texts and viewed as a relatively simple benign condition to the point where it is acknowledged that this is a complex condition involving not just the aortic valve but the aorta itself. The ACC/AHA 2006 Guidelines for Management of Patients with Valvular Heart Disease mention the issues with the aorta in those with bicuspid aortic valves. It is a great step forward in bringing greater awareness to the medical community that bicuspid aortic valves are more than just a valve issue, but there are still so many unanswered questions.
The full text of the following paper, published in the fall of 2003 by Dr. Gosta Pettersson and Dr. Richard Grimm of the Cleveland Clinic, is available online. I am going to list the link here. The subject is the use of the Ross in young adults. They make it clear that they are not speaking about children, which is a very different scenario.
Please be aware that there are graphic surgical pictures in the paper at the link that follows
http://www.hmc.org.qa/hmc/heartviews/H-V-v4 N3/6.htm
Regarding BAV and the Ross, this paper says "Patients with Marfan's syndrome or other connective tissue disorders have never been candidates for the Ross operation because of the presumed risk of autograft dilation and failure. David has raised the question of whether patients with bicuspid aortic valves beong to the same category(46)." It goes on to discuss conflicting findings in the medical literature. Figure 8 is a surgical picture of a BAV with a 4 cm aorta. Under the picture there is a question - "Does this patient have a tissue defect making him a bad candidate for the Ross operation?" Essentially, the question is not answered in this paper. Further along in the paper, patient selection is discussed. "Contraindications include Marfan's syndrome and other connective tissue disorders. Patients with bicuspid aortic valves might belong in the same category and are candidates only if they have no or mild aortic dilation." The paper ends by saying "Future generation bioprosthetic valves may well make allografts and Ross operations obsolete. However, limitations of present day bioprosthetic valves have kept allografts and pulmonary autografts in contention as an alternative."
People with Marfan syndrome have never been allowed to have a Ross procedure, so we do not know how much variation there might be in their results. Connective tissue problems in the body seem to have varying degrees of severity. I attended a Marfan meeting where there was a couple whose baby was born with a huge aorta, was on a beta blocker, and had aneurysm surgery at about the age of 2. At the same meeting was a man who was not even diagnosed until he was older - possibily around 60. So there are people on the extremes of all these conditions. The child would have had a very short life without surgery, while the other man went much longer than the typical Marfan before being diagnosed because of his eyes.
For Teri, who began this thread, according to this paper of Dr. Pettersson's, your husband with a 5.2 cm aneurysm would not be considered for a Ross procedure. I would encourage you to read the thread about DHCA and question surgeons about that as part of your research on aortic surgery.
I personally question the ability of anyone to know what will happen long term to the aortic tissue of someone with BAV. Visual inspection during surgery would not be enough to reassure me that my aorta will not cause a problem later. The only retrospective BAV study I am aware of, published by the surgical group in Milan, Italy, mentions that the aorta looked good at the time the bicuspid aortic valve was replaced, and yet when they checked on these people years later in many their aorta had clearly developed problems. Assuming that I was bicuspid and going in for surgery, I would be most comfortable personally with what this group from Milan recommends - but only if my aortic surgery is in expert hands. They recommend replacement of a seemingly normal and also a mildly dilated aorta at the time of the valve surgery. Below is the abstract from this group in Milan.
http://www.ncbi.nlm.nih.gov/entrez/..._uids=12440663&query_hl=1&itool=pubmed_docsum
Sometimes people might wonder if they can have both a Ross and ascending aortic aneurysm dealt with at the same time. Is it really even feasible to have a Ross and and the DHCA technique which removes the entire ascending aorta in the same surgery? The Ross procedure is understood to be a long and technically complex procedure. Aortic surgery with DHCA is extremely complex also. I doubt that both would be done equally well in the same surgery very often, and in a short enough time to be good for me if I were the patient. All things being equal, I would prefer DHCA and complete replacement of my ascending aorta above any other ascending aortic surgery technique.
As Rachel has indicated, Dr. Isselbacher's presentation, which was given just a couple months ago, presents the most recent understanding of bicuspid aortic valves. Yes, there is an underlying congenital connective disorder in those with BAV. The genetic reasons are being researched but have not been discovered so there is no genetic test for it, like they now have in Marfan syndrome. I would be interested in asking why a procedure that is not approved for the Marfan syndrome is thought to be acceptable for those with bicuspid aortic valves given today's understanding.
These are difficult questions. For all who deal with questions that today are the subject of debate, it is so important to be followed carefully regardless of the solution that is provided.
Best wishes to all,
Arlyss
