BAV patients who end up with aortic graph

[ctyguy]

Hi all...

Been a while since I have been on the site but this has always proven to be a tremendous resource when I've had a question or concern.

Today I had my 5 year checkup with my cardio. I had a BAV replaced with an ON-X valve. My doc told me all sounds good but he wants me to get an MRI to check my ascending aorta. He stated there is now evidence of higher rates of ascending aortic aneurism for those with BAV. Even after surgery. So of course now i am looking at statistics and am not real happy with what I am finding. I know this is just a routine test but honestly I thought I was clear of issues. Certainly I have made the most of it since my surgery living basically without any issues or limitations and being very active physically. I run a few times a week, eat basically what I want, and throw back a few cocktails/beers when the weekend roles around.

So I am looking to understand from others who had an AVR how many ended up with an ascending aorta aneurism down the road and if so how far down the road? Also has anyone been diagnosed with connective tissue disorder? And if so what were the symptoms? How was it diagnosed? I would also LOVE to hear from other BAV patients that have had no issues and have had their ascending aorta checked since surgery.

Scott

 

[Heart Of The Sunrise]

I had my ON-X valve implanted 8 months ago this week. At that time my aortic anueryism was repaired as well.

 

[mbeard]

Hi all...

Been a while since I have been on the site but this has always proven to be a tremendous resource when I've had a question or concern.

Today I had my 5 year checkup with my cardio. I had a BAV replaced with an ON-X valve. My doc told me all sounds good but he wants me to get an MRI to check my ascending aorta. He stated there is now evidence of higher rates of ascending aortic aneurism for those with BAV. Even after surgery. So of course now i am looking at statistics and am not real happy with what I am finding. I know this is just a routine test but honestly I thought I was clear of issues. Certainly I have made the most of it since my surgery living basically without any issues or limitations and being very active physically. I run a few times a week, eat basically what I want, and throw back a few cocktails/beers when the weekend roles around.

So I am looking to understand from others who had an AVR how many ended up with an ascending aorta aneurism down the road and if so how far down the road? Also has anyone been diagnosed with connective tissue disorder? And if so what were the symptoms? How was it diagnosed? I would also LOVE to hear from other BAV patients that have had no issues and have had their ascending aorta checked since surgery.

Scott

Dear Scott,

I am new to this site. I have a newly diagnosed enlarged ascending aorta, (4.2 4.5) , and biscusipd heart valve. July/August 2013. I am a newbie, as 'scared newbie' , is. I also hate to waste people' s time , because I am not scheduled for surgery...we are watching it. Every 6 months I go to Houston, Dr. Michael Reardon, and have a CT scan and Echo.

In saying that, I am anticipating an aneurism all the time...It doesn't seem fair to think about the fact that you could have another one, so I hope that someone will come on this website and say don't worry Scott.

mbeard

 

[ctyguy]

Mbeard..

I was freaking out when I had my diagnosis 5 1/2 years ago. It was my first surgical procedure other than tonsils as a toddler. What helped me was knowing the mortality rate was about 1%. I was 42 at the time and otherwise in pretty good health. The surgery itself went very smooth and I was home in 6 days, back to work in 6 weeks. Cleveland Clinic rocks! My valve was in very bad shape when diagnosed. I went for initial diagnosis to surgery in 15 days. My valve gradient was like 115 when normal is like 20. My cardiologist wouldn't do a stress test saying I was too high risk. I wouldn't even go for walks out of fear of raising my heart rate.

What may sound strange is that while it sounds terrifying I found it wasn't that bad. A bypass procedure is more invasive and a tougher recovery for sure. You get lots of drugs to manage the pain and as long as you can avoid sneezing and/or coughing you will be surprised at how quickly you recover. I was walking briskly on. Treadmill at 2 weeks post-op.

Do you have symptoms? I couldn't jog more than a few hundred yards without chest pain. That is what triggered me going to the cardiologist.

 

[Tdot]

Hi ctyguy

I met with a surgeon on Saturday after a referal from my cardiologist who explained a lot to me. I have BAV with a dilated aortic root at 44mm. Been that way for some time. The surgeon explained that they have found people wih BAV are also missing some fibrillin1 in their body. People with marfans have none and are likely prone to aneurysms. Firbrillin1 helps bind your tissue together and if it's missing in whole or in part it could make your aorta weaker. He also saiid fibrillin 1 is the geneic marker that causes the valve to split into three leaflets during development Of the fetus and is the root cause for a BAV. Therefore the correlation to aortic aneurysms and the need for screening.

However there are all of people hear who have BAV and no issues with their aorta. I had an MRI and my aorta is ok but the root is dilated. Everyone is different so it doesn't mean you will have this issue.

 

[AZ Don]

So I am looking to understand from others who had an AVR how many ended up with an ascending aorta aneurism down the road and if so how far down the road? Also has anyone been diagnosed with connective tissue disorder? And if so what were the symptoms? How was it diagnosed? I would also LOVE to hear from other BAV patients that have had no issues and have had their ascending aorta checked since surgery.

BAV is suspected to BE a connective tissue disorder - see Tdot's answer. That's why the criteria for surgery for an aneurysm is sooner for those with BAV. Unfortunately having your valve replaced does nothing for your risk for aneurysm. Everyone is different though, many have no aneurysm, occasionally people have more than one. Arnold Schwarzenegger had a bicuspid aortic valve and never had an aortic aneurysm.

 

[mbeard]

S,

I do have constantly pounding heart. I am going to my local cardiologist tomorrow to talk to him about it. (I have been to the ER once already thinking I am having a heart incident). I am scheduled to go to Houston in January for my first 6 month checkup . My diagnosis was in August 2013.

My constant worry is that I don't have a surgeon in my hometown. What if I have an emergency? It's on my mind.

Bicuspid heart valve, enlarged ascending aorta, 4.2,4.5.

 

[ottagal]

Hi all...

Been a while since I have been on the site but this has always proven to be a tremendous resource when I've had a question or concern.

Today I had my 5 year checkup with my cardio. I had a BAV replaced with an ON-X valve. My doc told me all sounds good but he wants me to get an MRI to check my ascending aorta. He stated there is now evidence of higher rates of ascending aortic aneurism for those with BAV. Even after surgery. So of course now i am looking at statistics and am not real happy with what I am finding. I know this is just a routine test but honestly I thought I was clear of issues. Certainly I have made the most of it since my surgery living basically without any issues or limitations and being very active physically. I run a few times a week, eat basically what I want, and throw back a few cocktails/beers when the weekend roles around.

So I am looking to understand from others who had an AVR how many ended up with an ascending aorta aneurism down the road and if so how far down the road? Also has anyone been diagnosed with connective tissue disorder? And if so what were the symptoms? How was it diagnosed? I would also LOVE to hear from other BAV patients that have had no issues and have had their ascending aorta checked since surgery
Scott

Hi Ctyguy,
Good to hear from you and glad life has been good post AVR. I had AVR 4 years ago and 6 months ago, my echo showed a 4 cm aneurysm in my distal ascending aorta. You can imagine what a surprise this was for me so soon after. I am also getting an MRI to confirm the finding as echos are not always accurate. I think I am in the minority as I recently posted a thread looking for members who got this similiar diagnosis POST AVR and I only had a few replies. So, try not to worry about the what ifs. I couldn't find any statistics on the percentage of those who develop an aneurysm post AVR. Are you getting the contrrast with your MRI? I am still hoping my echo was inaccurate and the MRI shows no aneurysm.
Do keep us posted. I am sure all will be fine. Good to see you post.

 

[clay_from_nj]

Bicuspid valve, stenotic and regurgitating; ASD (atrial septal defect - "hole in the heart") repaired in childhood. No aorta issues at all, no blockages. Aside from the heart failure caused by the valve, no cardiac or vascular issues at all. No Marfan's, no diagnosis of any connective tissue disorders.

 

[OldManEmu]

ctyguy I had a 4.7cm aneurysm at the time of my first surgery in 2004 and it couldn't be repaired because my heart failure was so bad the surgeon estimated I would have had a <50% chance of survival if this was attempted. The aneurysm stayed the same for 6 years until it enlarged triggering an ascending aorta replacement and an AVR redo. My tissue valve had a small increase in valve pressure over the 6 years and the surgeon estimated it was another 3+ years from needing replacement, however it was replaced at the time of the ascending aorta repair to spare me another surgery in only a few years. I hope this is the last surgery for 10+ years.

 

[ctyguy]

I had my MRI today, needless to say I am a bit restless. Thanks to those who posted I will post after I hear from my doc.

 

[neil]

as many have said life is full of ifs, many dont have any problems with aneurysms ,some do, hopefully good news for you today

 

[aussigal]

I had the Bentalls procedure aortic valve and ascending aorta replaced in one simple op...they did tests on my tissue at the time and it was confirmed that the tissue had undergone changes consistent with a connective disorder (myxomatous was the word they used). I have dodgy tendons and joints so it all seems to fit.

 

[nightingale]

Hi ctyguy, I haven't been on this site for a while, but saw your posting and had to answer. I had a



Hi ctyguy, I haven't been on this site in a while, but saw your posting and had to answer. I had an AVR 7 years ago for a BAV. Thought that was the end of it until a couple of years ago when my cardiologist said my aortic root was dilated and they would keep an eye on it. This last year, her told me it was time to get an MRI, and sure enough I had an ascending aortic aneurysm that was previously not there. Long story short, had my AAA repair and graft in September. I was very upset when I got the news that I would have to have another surgery, but now I'm on the other side of it and life is pretty much back to normal. I wouldn't have chosen this path for myself, but here I am, so I do the best with what I have. I'm back to running several days a week, and generally taking good care of myself. Please let us know what your MRI showed. I'm hoping the best for you.

 

[ctyguy]

Hi ctyguy, I haven't been on this site in a while, but saw your posting and had to answer. I had an AVR 7 years ago for a BAV. Thought that was the end of it until a couple of years ago when my cardiologist said my aortic root was dilated and they would keep an eye on it. This last year, her told me it was time to get an MRI, and sure enough I had an ascending aortic aneurysm that was previously not there. Long story short, had my AAA repair and graft in September. I was very upset when I got the news that I would have to have another surgery, but now I'm on the other side of it and life is pretty much back to normal. I wouldn't have chosen this path for myself, but here I am, so I do the best with what I have. I'm back to running several days a week, and generally taking good care of myself. Please let us know what your MRI showed. I'm hoping the best for you.

I got my results the day before thanksgiving and I am in the clear, at least for now. My cardiologist said we will check again in 5 more years. I am relieved but realize another procedure could be in the works down the road. I am hoping to procrastinate long enough so that the technology advances to be a less invasive event. Certainly if I have to have it then I will want it done straight away.

I am using this as a reminder to myself to make the most of every opportunity that comes my way as you really don't know what is around the corner in life.

 

[Superman]

ctyguy,

Late reply, but I had an AVR in 1990 just before I turned 18. Almost 19 years later, I had a second OHS for an aneurysm repair. I had them update my hardware while they were digging around. Hopefully that's it for a while. I had no symptoms and it was a routine echo that showed the dilation. This led to a CT scan, then surgery. Never had any discussion of aortic root measurements until a month before my second surgery. It seemingly ballooned all at once. I had them pull prior echo results and compare measurements and it was always around 3 cm up until that one.

My concern is primarily the possibility of a systemic connective tissue disorder. I guess I keep waiting for what's next and wonder if the odds are greater in patients like myself for other aneuryms (brain in particular). Reading Tdot's post, it doesn't make any sense to me that this would all somehow be isolated around the heart only. I would like a CT or MRI of the brain, but don't know that I really want to push for it.

 

[HopefulHeart]

Hey there Ctyguy.....

I've heard of some people getting an aneurysm after having a BAV repaired. My Cardio told me that the gene that codes for the aortic valve also codes for the section of the ascending aorta right above the valve. So if there is a hiccup with that gene that caused the BAV, the connective tissue right next to it may well be compromised as well leading to an aneurysm. That's how it was explained to me. I have both a BAV and an ascending aortic aneurysm at 4.2 cm just above the valve. I have not had any surgery yet as my BAV is in good shape, I have no symptoms and my aneurysm has not reached the size in which they operate. Glad to hear you are in the clear after your MRI. By the way, when the time comes for me to have surgery, I plan on going to Cleveland Clinic. I'm glad to hear your experience there was good.

 

[TheGymGuy]

When I first met with my surgeon and we discussed BAV, and a bit of aneurysm on my ascending aorta he did tell me that there was a genetic connection between BAV and weakened aortic tissue. I think elsewhere on this board there are posts with more details and links to sites like NIH, etc. I can try to look some up if you'd like.

My surgeon replaced the aneurysmic (is that even a word) tissue and went all the way up the the hemi-arch of the ascending aorta to increase odds of us not having to do this again. The only part thats original for me is the aortic root, the rest of it up to hemi-arch has been nicely replaced.

Now, here is the good news. They can catch changes in your ascending aorta especially area close to the valve and most of the ascending aorta with your yearly echo. Also, the connective tissue disease is a crapshoot, just like BAV needing replacement is a crapshoot. Some people have BAV and never need it fixed. Case in point my grandma. She had BAV and never had it fixed. Died in her 70s from Cancer and her heart was still working fine. So, with aneurysms similar thing would apply, if your aorta was free of it when they were in there looking around and fixing BAV, who is to say it will have an aneurysm. My vote is that you keep on doing what you are doing and do not skip your yearly checkups. If there is any evidence of anything happening they will catch it before its too serious of an issue.

 

[Agian]

I was told that the ascending aorta and the valve have the same embryological origin. The arch can be affected by the aneurysm spreading south. But the connective tissue beyond the ascending aorta is normal in those with BAV. Basically, what this means is that you are at no higher risk of an aneurysm than everyone else, beyond the repair. The connective tissue problem is not widespread. This is how it was explained to me. However, BAV may be associated with aortic narrowing, further downstream. You either have it, or you don't. It's not something that develops in adulthood. If your aorta is narrowed, the blood pressure in the upper part of the body is higher than the lower part. High blood pressure, of any cause, is associated with another set of problems.

PS, a graft will last a lifetime.