Reinforced Aorta

[BarbJean]

I just have a question, but hope I am not taking this thread off topic. When you all mention connective tissue disorder in relation to bicuspid aortic valves, are you referring to Marfan as a possibility? I am just curious, because my son has Marfan but does not have BAV. His aortic root was measured at 4.59 at last echo but he has no leakage at all. My friend's son was always told he probably had Marfan. He had aortic surgery a year ago to replace his BAV. By the time he had the surgery his aortic root was measuring about 7cm. Phew! He could never be officially diagnosed with Marfan because he does not have dislocated eye lenses like my son does. Now that you all talk about BAV paired with connective tissue disorder, his condition makes more sense. He may not have Marfan after all, but my son definitely does. Thanks, Barb

 

[Maryka]

As stated by others, I would choose to have the aorta reinforced/fixed while the surgeon is in there. Every new surgery is a new risk. You will need to go back for more surgeries if you do not take this oportunity to fix the aorta.

BarbJean: I guess you remember that I was diagnosed with Marfan Syndrome 20 years ago and then down-graded to a diagnosis of "Marfan-like Syndrome" and finally, after a trip to Stanford, was found to have BAV with an aortic aneurysm. A lot more of the population has BAV with some connective tissue involvement than have Marfan syndrome. Some people, like me, have lots of other connective tissue problems with our BAV. It seems that there is a sub-group (without a nice and neat name like Marfan Syndrome) who have a specialized syndrome with connective tissue problems. (On the other hand, there are lots of BAV people who do not have connective tissue involvement and perhaps many more people who never know they have BAV and live a long life without heart or connective tissue problems.) Oh, and then there are people like my friend Carol who have aortic aneurysms but have perfect hearts. It's complicated.

 

[cbdheartman]

I'd have to agree with my friend Maryka about not wanting to do repeat surgeries if at all possible. I can tell you (and those I drove crazy here) that the psychic weight of knowing you have an aneurysm -- which you do, I think anything over 4.0 cm is consider an aneurysm -- is really weighty and saps you. I am so glad that I had surgery last summer. I was faced with a different situation. Good valve situation with an aneurysm on the borderline. In surgery, the measurement ended up being 5.0 cm even though the scans were showing smaller. I don't know if that is typical, but definitely keep that in mind and perhaps ask you surgeons about that.

Also, to piggy-back on something Maryka said about connective tissue issues. Working with the wonderful Dr. Dietz at Hopkins, I have learned that there are those with BAV who have connective tissue problems. Doctors -- and many still think this is the case -- used to believe the changed hemodynamics from the leakage caused the dilation. That theory has been put aside and now they believe it is basically a genetic problem that causes the equivalent of loose or weak glue in the tissue leading to expansion.

Also, as Maryka mentions, you can have a normal valve and connective tissue issues. So for instance, I've been told that my children have a 50% predisposition to getting aneurysms. I don't totally understand this, but this does not mean that they will develop aneurysms but that they will be in a subclass that are predisposed to them. And my understanding is that this predisposition does not correlate to BAV. They could have great valves, but aneurystic tissue. They could also conceivably have bad valves and no connective tissue problems. Amazingly only 1/3rd of those with BAV (without connective tissue problems) ever need to have their valves replaced.

 

[john79t]

All the research I read said that the wrap has greatly improved and does limit future increase. I went with the wrap. I have a much greater fear of rupture like Ross had that any possibility of infection. Infection was not a significant problem in any of the research I read.