23 years old, first heart surgery, brittle bones, very scared

[kirstinmariec]

I was diagnosed with aortic insufficiency about a month ago and I am scheduled for an aortic valve replacement June 30th (mechanical). I am 23 years old and the news was a complete shock to me. But my cardiologist said I've had it for years and I am thankful they finally heard the murmur. I believe the diagnosis was caused by a bone disorder I have called Osteogenesis Imperfecta. This is a brittle bone disease but it also causes weakened tissue (including the heart). I only have a mild form of it but I am still more fragile than a "normal" person.

This concerns me a lot when it comes to them needing to spread my ribcage, because I break more easily and I have a slightly barreled chest. I cannot find any research on my condition and OHS. I am very scared and anxious all of time. I am reaching out on this forum, hoping someone has ANY information or advice. I am so worried that more complications come along with my condition. But I just don't know.

I do believe that my surgeon has experience with patients who have OI, but because I haven't consulted with him yet I don't know any details. I am still very worried about how my body will be handled during the procedure. I will be meeting him and having pre-op early June. Other than this new heart diagnosis, I am a fairly active, healthy, and normal 23 year old.

I would appreciate any prayers and encouragements as well!
Thanks everyone!

UPDATED:

Thank you everyone for your replies.

Just a quick update: I had my pre-op appointments this week and met with my surgeon (Lars Svensson, MD, Cleveland Clinic). I believe to be in good hands, as he is a very experienced surgeon and he has some experience with my condition (OI). However he mentioned his biggest concerns are the fragile bones and heart tissue. We discussed and decided on minimally invasive surgery (mini-sternotomy) and replacement of the aortic valve with a mechanical valve. The minimally invasive surgery means that he will only split open about 1/3 of the sternum (from top down) to reduce the risk of bone fracture. What worries me the most is that he will not know the how fragile the heart tissue is until he actually opens me up. If the tissue is too fragile, it may be too difficult to hold the stitches which holds the new valve in place.

I am trying to stay calm, because now it is out of my control. Not knowing is the hardest part. 25 more days.

Please again, if anyone has experience or knowledge of Osteogenesis Imperfecta and OHS. Please share with me. Thank you everyone!!

 

[Paleowoman]

I have osteoporosis which is also "brittle bone disease" and has a high risk of fracture and when I had OHS in January this was one of my concerns. I was really worried about the way my sternum and rib cage would be spread apart and my body would be manipulated during surgery - I have a depressed sternum which I would have thought would have meant even more manipulation. I made it clear to the surgeon that I have osteoporosis and that I was worried about the fracture risk. I also made sure the nursing staff were fully aware of my osteoporosis so that I would be 'handled' supportively and carefully.

So, although there doesn't appear to be any information about fracture risk online, if I were you I would just make sure the surgeon and the nursing staff are all aware that you have an increased fracture risk.

I didn't get any fractures during surgery and I was assured that they routinely do this sort of surgery on frail elderly women and so they are always very careful

 

[AZ Don]

I suggest that you talk to your surgeon and ensure that he/she understands the extra risk of complications that Osteogenesis Imperfecta may present during OHS. It may be useful for your surgeon to talk with your Dr. regarding OI. I googled OI and OHS as well as OI and Valve Replacement and found references to a number of case studies. I saw it mentioned that people with OI may be at risk for "hemorrhagic complications considered to be related to tissue friability". http://www.atcs.jp/pdf/2002_8_1/51.pdf. It sounds to me like the extra risks may be mitigated to some extent by the surgeon having an understanding of your condition and treating the tissue (and bones) gently. You say your condition is mild so perhaps this means your extra risk of complications is mild as well. OHS is generally very low risk for someone that is young and healthy. Many people on this site will tell you that it was not as big a deal for them as expected - that was the case for me.

I see that you are in Cleveland which may be fortuitous for you as I believe the Cleveland Clinic may be the best hospital for heart issues in the country. Also, they are often doing valve replacements with a less invasive procedure than a full sternotomy, and that may make a lot of sense for you. I highly recommend you consult with a surgeon there. If you just call them up and explain your condition they can probably find the best Dr. for you to be talking to.

Good luck.

 

[knotguilty]

If all you are having done is an aortic valve replacement, I would think they would do a minimally invasive technique where they go through the ribs at your right upper pec muscle. That is what I had done. I think they actually do cut a rib to facilitate entry. But for me, and you have youth on your side, I bounced back rather quickly. You will be in good hands and you will be fine.