Dilated Aortic Root - Terribly Nervous

[pearjas]

Hi everyone,
Though I know my situation could be much worse, I am not doing well as I have been overwhelmed with anxiety since the 19th of this month.  I received news that my aortic root was dilated at 4.4cm without any further explanation.  I had an echo in 2012 which stated it was 3.5.  To make a long story short, I had a CT scan, which showed it to be an even 4.0 and I seen my cardiologist for the first time yesterday who stated that it is 4.0 but commented he looked at six or seven images and he seen nothing larger than 3.9.  So I did receive good news at the cardiologist yesterday, however…

I’m a 32 year old male, around normal weight or underweight at around 133 pounds or so.  Before all this came up, I was going to the gym nearly every day, and had started on fairly extensive weight training as of late last year.  I have been diagnosed with a connective tissue disorder as well – Ehlers Danlos Hypermobility Type.  Though I have that, I’m obviously one of the lucky ones in that it doesn’t greatly affect me or what I can do.  I rarely have complications with joints, etc though am clearly hypermobile.  

It was in 2012 I requested my doctor at the time to do an echo, and it was me again that asked for another one this month, as I keep up to date on most of the reading about Ehlers Danlos, and given it’s still ‘unknown’ to doctors in many cases.  So for peace of mind, coincidentally enough, I wanted this second echo done.  Things just haven’t been the same since.  

I wasn’t overly impressed with my cardiologist yesterday, though he did seem to know what Ehlers Danlos was, etc.  He said that I more or less do what I was before, but not to “go overboard” and we’d do another scan in a year.  Everything I read (or nearly everything) states this is likely to grow anywhere from an average of .07 to 1mm a year.  This would mean I’m eventually going to need an operation.  I don’t know how much, if any, stopping weight lifting would help.  I am just lost about what to do.  While I know no one can predict this for me, I just feel like I should know more than I do.  Or be more confident that I know what I should be doing.

The biggest fear for me is the surgery, which I’ve never had.  I’ve always feared anesthetics (ever since I had a reaction to one in the dentist chair many years ago) and obviously if your aortic root needs to have surgery, that’s a major operation.  I’ve not read about any endovascular methods ever being used for the aortic root despite being available for other areas.  Am I just missing this?  While I don’t know if I’d ever do endovascular, knowing it would be an option surely helps.  I’ve done a lot of reading on “TEVAR” surgery, but nothing lists the aortic root, as it always seems open surgery is the only option.  

Does anyone else have any experience with this?  I have no family history of aortic problems.  I am the most affected by hypermobility in my family, but don’t know what bearing that has when it comes to a dilated aorta.  Am I destined for surgery later down the line?  Do these things almost always grow large enough for surgery?  Any thoughts or advice?  : (  I can't explain how much I've been stressing about this and I fear it's going to be the entire year long wait until the next test.

[achillea]

I agree that you would probably benefit from talking with a cardiologist with special knowledge--as well as a rheumatologist with a good understanding of cardiology!

However, some scholarly papers out there indicate that the factors that cause aortic root enlargement are more varied than once thought, and having E-D is not necessarily a road straight road to surgery, especially if you do *not* have the vascular type of E-D.  Check out the link here, for example:

http://www.jpeds.com/article/S0022-3476(11)00295-2/fulltext

[skydnsr]

You have a lot of questions about your Ehler Danlos Syndrome, appropriately so, and I think the answers to your questions need to come from a cardiologist.  If you do not have confidence in your current doctor, I would try to find a cardiologist who has a particular interest in EDS.  I looked at your profile and saw that you are located close to Kansas City MO, which is a large metropolitan area.  You should have an excellent chance of finding an EDS doctor in the KC area.  There will not be a cardiologist who specializes in EDS only, so you will be looking for someone who has a more general interest in connective tissue disorders that affect the heart, such as EDS, Marfans, bicuspid aortic valve syndrome, Loeys Dietz, familial thoracic aortic aneurysm, etc..  In St. Louis, there is a center that would fit you to a "T:"  the Barnes Jewish Hospital/Washington University Center for Thoracic Aortic Disease.  If I were you, I would consider traveling to St. Louis, at least one time, for a consultation.  If that is impossible, then I might call the Center in St. Louis and ask if they can refer me to a KC-area cardiologist who has a special interest in EDS.  You could also check with EDS support groups, to see if any EDS patients can recommend a good cardiologist in Kansas City.  A quick google revealed that there is a St. Louis area EDS support group that has a FB page, so you might check with them.  Good luck.