Lukkychrm42 Posted February 22, 2007 Report Share Posted February 22, 2007 Hi friends,I saw a new cardio yesterday, and while it seems he might be helpful in the POTS arena (apart from wanting to refer me to Dr. Grubb), he seems to think that with EDS I'm at a higher risk for aortic dissection. Am I right in thinking he's probably confusing EDS with Marfans? Unless I had the vascular type, which I don't. He wants me to have some sort of angiogram to check on my aorta. Anybody have a clue?Thank ya muchly,Megan Quote Link to comment Share on other sites More sharing options...
MightyMouse Posted February 22, 2007 Report Share Posted February 22, 2007 That's a risk associated with EDS-Vascular (was formerly called type IV). See EDNF.org for types of EDS. Marfans does have some overlap. My neuro thought I was higher risk for brain annyeurisms, b/c he had a colleague with EDS who died from a brain bleed when they were residents. I explained that she was likely type IV, and I'm type III. But, he did have me get an MRA to make him feel better. Nina Quote Link to comment Share on other sites More sharing options...
persephone Posted February 22, 2007 Report Share Posted February 22, 2007 I've been told by a leading Rheumatologist that there IS a connection between EDS and aortic dissection. In fact, they thought I was having one last summer when I got admitted to hospital for 7 weeks, tho it turned out to be a false alarm. I also have EDS III rather than IV. Quote Link to comment Share on other sites More sharing options...
Lukkychrm42 Posted February 23, 2007 Author Report Share Posted February 23, 2007 Actually I think you might be right- I just checked the EDNF page and while certainly the vascular type seems at most risk of problems with the aorta, it said there was such a heterogeneity among the EDS types that screening for aortic dilation should not be limited to type IVs. Hmm. I'll let you know how it goes! Quote Link to comment Share on other sites More sharing options...
mkoven Posted February 23, 2007 Report Share Posted February 23, 2007 I think that now they are finding more crossover between the different types. On the ednf website, they are saying that 25% of those with type 3 and 33% of those with classical have some aortic dilation--which should be monitored, because it may put someone at risk of rupture. I've been told to have yearly echocardiograms, even though I'm either hypermobile or classical, not vascular. It makes sense, as we're talking about tissue fragility from faulty collagen. Quote Link to comment Share on other sites More sharing options...
anna Posted February 24, 2007 Report Share Posted February 24, 2007 Hi Guys,We are EDS3 kids and I and have echos to check for MPV and dillation of the assending aorta. You might find a paper on EDS 3 by Howard P Levy, MD PhD quit useful, go to WWW.genetests.org and do a search for EDS 3 by the afore mentioned Author.Anna Quote Link to comment Share on other sites More sharing options...
goldicedance Posted February 24, 2007 Report Share Posted February 24, 2007 Is aortic dissection the same as an aortic aneurysm?Dr. Grubb said I had EDS hypermobility. Because my mother had an aortic aneurysm, my local cardiologist had me have an abdominal scan where they can see/find aneurysms. I did not have any.Moreover, I believe the University of Maryland has a study on EDS and aortic dissection.To tell you the truth, we worry about so many things that probably have a very low likihood of happening. I am more concerned when I drive on the beltway or interstate of accidents. I think sometimes we get so consumed by our health that we worry too much over little things.Lois Quote Link to comment Share on other sites More sharing options...
mkoven Posted February 24, 2007 Report Share Posted February 24, 2007 since the testing for aortic dilation is not a big deal (not very invasive, dangerous, painful)--echo cardiogram and/or ultrasound, I think many docs feel it's worth it. If heds has 25% rate of dilation and ceds 33%, that's not trivial. Again, dilation or aneurysm is not the same as rupture-- it just raises the risk, as the walls are stressed/stretched. I think monitoring is not a bad idea, if you have a connective tissue disorder, to make sure there is no dilation, or if there is, that it is not getting worse. I believe most docs will let patients go to a certain size dilation before they intervene surgically. If you are dilating, typically the recommendations are to avoid hypertension or spikes in blood pressure that stress the weakened wall further-- so no lifting of heavy weights, etc. I have no dilation, but will continue with annual screening.And from what I understand, aortic dissection is not something to be messed with. Once the aorta ruptures (actually rips, not just stretches), the outcomes are often not good at all. That is what John Ritter died of (without connective tissue disorder). Older folks with hypertension can rupture--my boss died of this in his early 60s. One of my best friend's mother had this happen, and she did survive though. Quote Link to comment Share on other sites More sharing options...
cardiactec Posted February 24, 2007 Report Share Posted February 24, 2007 hi,an aortic aneurysm, or aneuryms of any kind, are weakening in the lining of a vessel causing it to increase in diameter. a dissection is when that aneurysm tears open the lining of the vessel, causing the vessel blood filled cavity to "leak" blood into interstitial space - causing the patient to bleed to death (if not caught on time to fixed the torn vessel. i have heard, especially with sinusus of valsalva or ascending aortic root dissection, that unless you are on the operating room table and wide open for surgeons to get ahold of the dissection, you chances of surviving an ASCENDING aortic root aneurysm that has dissected are slim to none....so pretty scary stuff. my mother has an ascending aortic root aneursym that she didnt even know she had (i was actually the one to find it while i was doing my clinical rotation for my cardiovascular degree).........the docs are blaming it on "HTN" but her BP isnt that high, so i'm suspiscious that there is either EDS or marfan in our family. and my mom's mom's mom died when she was only 28 -- bent over to tie her shoe and stood up and died (sounds like an aortic root rupture to me, they blamed it on a stroke but back then, they blamed everything on a stroke and didnt have the technology to access the aorta either, so dont really know) ..........i fit the bill to a T with many features of marfan (very tall, lanky, arachnodactly, pectus excavatum to a mild degree, MVP, TVP, hypermobile).............i will be seeing a genetisict in two weeks for further evaluation.......... Quote Link to comment Share on other sites More sharing options...
goldicedance Posted February 24, 2007 Report Share Posted February 24, 2007 I do get an echo every other year because of my hypertension.I did not mean that an aortic dissection is trivial. What I meant to say is you do the necessary testing for the condition, take the doctor's advice about what to avoid, and then get on with it. If you focus too much on this, you lose sight of other things. At this point in my life, I am more worried about my breast cancer and the future. POTS is secondary.Also, re Marfans. I do not have it. I do know it is a serious condition. I once tried Sandostatin for my POTS. I did very well on it until I got really high blood pressure and gall bladder problems. Somehow, I think that Sandostatin is used to treat Marfans. If that is the case, maybe you might inquire about Sandostatin. Quote Link to comment Share on other sites More sharing options...
Lukkychrm42 Posted March 3, 2007 Author Report Share Posted March 3, 2007 Hey everybody.I had my CT scan yesterday- the IV was lots of fun and left a nice big blood blister, the contrast fluid was freaky feeling, but it only lasted about 2 minutes. While I was in there the doctor called and said he wanted to discuss some medicine changes... by the time he called back 3 hours later (6 pm), he said he didn't want to change angthing yet, because they found a "mass" in my mediastinum (behind my sternum, between my lungs and heart), and he said he had already talked to some doctors on my behalf who wanted to squeeze me in, and they would call me ASAP on Monday with an appointment time with a pulmonologist and to decide on a biopsy and treatment. Obviously they won't know if it's anything malignant until a biopsy, but right now based on the CT scan, it did not appear to be a cyst or calcium deposit or whatever. Anyway, I guess that's my excitement for the week- something to keep me twiddling my thumbs until Monday morning, anyway. Don't worry though, I'll let you know what's up. Because I won't get my DDAVP until it's out! Grr!! Oh sorry, BTW my aorta is fine. Quote Link to comment Share on other sites More sharing options...
cardiactec Posted March 3, 2007 Report Share Posted March 3, 2007 megan, did the doc say what he thought it was? have you been short winded at all? had your doc primarily ordered the CT just to **** your aorta for possible connection of connective tissue disease? if so, how odd that they found this mass when werent even looking for such a thing....i hope the biopsies show nothing serious.i'll be thinking/praying for you. keep us posted and hang in there.angela. Quote Link to comment Share on other sites More sharing options...
Lukkychrm42 Posted March 4, 2007 Author Report Share Posted March 4, 2007 Hi,Yeah he just threw the CT in for the aorta bit. I've been having a lot worse symptoms lately, including more breathlessness, more dizziness, worse balance, weakness, and more malaise- but any could have been attributed to POTS, too. I just have to get through tomorrow and then will hopefully know more!! Anyway, I'll let you know what's up. THanks!Oh man I am so impatient! I've been zoned out all weekend! Thank goodness it's nearly Monday!!! Quote Link to comment Share on other sites More sharing options...
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