Questions from a person with BAV and Root Aneurysm

[cbdheartman]

Hello there,

I am new to the forum. A bit of background: I am 31-year old male, 6 ft, 4 in, tall, diagnosed with BAV at age 3; I have mild/moderate aortic insufficiency. My reports say I have LVOT + 2 aortic insufficiency, no stenosis.

In recent years, we've watching the aortic root and ascending root. Unfortunately, we've followed it with a number of different tests. In 2006, a CT-Scan showed the root at 4.8 cm and the ascending aorta by the right pulmonary artery to be 3.9 cm.

A regular chest MRI last year showed these measurements at 4.5 and 3.7.

This year I was sent for a cardiac MRI and this report showed at diameter of 5.2 cm at the root and 2.9 at the right brachiocephalic artery (which I assume is the right pulmonary artery?).

A surgeon last week who viewed the MRIs himself said, despite the MRI reports, that he did not believe there had been much change in the past year and believes I am 5.0 or 5.1 at the root rather than 5.2. (The change from 2006 would be, he said, consistent with a typical 1 mm a year change.)

I have now visited with 2 surgeons and sent my materials to a doctor at the Cleveland Clinic. The first surgeon said to have surgery within the year. The second, who does valve-sparing surgery, said he thought I was more in a gray zone and does not recommend surgery until my root reaches 5.5 cm (though surgery now is not unreasonable according to him). The Cleveland Clinic's assistant said that he doesn't recommend surgery yet either, but would like me to come in for an evaluation.

This gets me to my questions and I will throw a few out there.

1. Does anyone know why surgeons would be recommending that I wait? Is it because the risk of surgery is higher than the risk of the aneurysm dissecting or rupturing?

2. Why would I wait? I am weighed down with thoughts of the aneurysm bursting among other things.

3. I am getting different advice on what limits to adhere to in my daily activities. My cardiologist said to refrain from exercise and lifting (obviously I've been avoiding heavy weight lifting for years anyway). The first surgeon said not to get my heart rate up, but another surgeon said I could do 20 minutes of light running on the treadmill. I am erring on the side of caution right now, but I was wondering if anyone else faced with similar situations could provide advice.

4. Do aneurysms such as mine ever stop growing or can they ever repair themselves? If I wait on having surgery, is there any hope that the aneurysm will simply stabilize and not continue to grow? Or am I just in a waiting game where some day the aneurysm (unless something bad happens) will reach the magic number of 5.5 cm and then I will have to have surgery? (If that is the case, again I wonder why I shouldn't go ahead and have it now.)

5. One possibility I have heard is valve-sparing surgery. Obviously the valve can deteriorate over time: so is this a reason to go forward with the surgery now rather than later?

6. Does anyone have any experience with this sort of surgery at the Washington Hospital Center or Johns Hopkins? I am also considering the Cleveland Clinic, but that is farther away from where I am now.

7. Does anyone have general thoughts on the situation I am facing, the David-valve sparing procedure, and where to have such a surgery?

8. Does anyone have any personal experience with one of these three surgeons: Ammar Bafi, Washington Hospital Center; Duke Cameron, Hopkins; Lars Svensson, Cleveland Clinic.

I welcome any and all thoughts. I am married with two small kids and this latest development in my health seems to be paralyzing us as we make decisions. It almost seems worse to be in the gray zone than to be in a position to say just do the surgery.

 

[ottagal]

Hello cbdheartman,

I wanted to wish you a very warm welcome to the VR family. This is a great place to get support! Know that you are not alone and getting this news can be 'paralysing' to say the least. As many will attest, the waiting is the worst part. I am sorry I can't answer your questions, but am sure others will chime in soon to help you out.
Best wishes

 

[Reddog]

BAV Surgery

BAV Surgery

There are several different criteria that doctors use to determine when to replace an aortic aneurism. One basic criteria is 5 cm or more at the root (smaller if you have Marfans). Another relates to the size of the ascending aorta vs. the descending aorta. Another relates to the ratia of the aorta diameter to body area. Another criteria is how fast the aneurism appears to be growing. So you are going to get different advice from different doctors. It would be wonderful if everything were definitive, but hey, this is medicine, and believe it or not, they don't have a whole lot of data on BAV patients even though it's not an uncommon problem.

A few answers to your questions:

1) I was told that aneurisms never really get smaller. You may get a smaller measurement with one test due to the error intrinsic in the measurements, but the aneurism isn't getting smaller.

2) If you are in the grey zone, and you are weighed down with thoughts about the aneurysm bursting, I'd get the surgery done sooner. As many point out here, the waiting is the hardest part. Or so I'd like to think, since my surgery is scheduled in about a month!

3) Valve sparing is a very reasonable choice for someone like you if they can address the aortic insufficiency. That's a question for the surgeon. It is great that you don't have stenosis, because that would pretty much rule out a repair. Since bicuspid aortic valves do become stenotic in your 60's or 70's, another operation will likely be necessary at some point, but if you would like to avoid coumadin, a David-type valve sparing operation may be optimal for you.

Now here's a bit of extra background about me, because my case is very similar to yours. I am 6' 2" tall, and I have a bicuspid aortic valve which is 5cm at the root and 5.4cm a bit higher up. It has been stable for the last 7 years at least. This also puts me in the grey zone. However, my cardiologist is one of the world's experts at BAV (Dr. Otto at UW), and she is recommending that I have the surgery now. I also met with two great surgeons, and they said that surgery this year would be a really good idea.

Here is the logic from the surgeons that convinced me. They said that I have a 1%-8% chance of an aortic dissection each year, given the size of the aorta, though more likely on the 1%-2% side because it has been stable so long. Keep in mind that the statistics aren't bulletproof, but they still help me to think about the problem. The operation, when done by a great surgeon, has roughly a 99% success rate (and needs to be done only once if you are getting a mechanical replacement... and maybe twice if you go the repair route). So given these stats, the decision to have the surgery soon makes a lot of sense. 1-2% chance of death per year vs. 1%-2% chance of death once.

Like you, I have several young children (9 and under), but I'm somewhat older than you (46 years old). Right now, I think that it's most likely that I'll choose the David Procedure. Why? Well, my bicuspid aortic valve is in pretty good shape, and though many on these forums have no issues with coumadin, I have some ancillary health issues that might make coumadin more of a challenge for me. My surgeon, Craig Miller at Stanford, is one of the greats at valve-sparing operations, and he thinks there's a good chance that I'll get 15-25 years out of my valve, even at my advanced age. And who knows... they may find a drug to halt the stenosis of BAV valves or use a catheter-delivered replacement when the time comes.

Aneurism repair is much more difficult than valve replacement, and valve-sparing operations add complexity on top of that. If you choose to go the valve-sparing route, choose a doctor that has a reputation for this. I think there is one at the Cleveland Clinic. Craig Miller at Stanford is another option. And if you want to make the trip up to Canada, I think Tyrone David is still working his magic up there.

On the other hand, if you don't think you'll have issues with coumadin, you might choose to go with a mechanical valve, and get the job done once and for all.

Best of luck with your decision, and welcome to the online community. If you have any questions, feel free to send a private message, and I'll provide any additional info that I can.

Regards and Best of Luck,
Reddog

 

[briansmom]

Welcome to the group cbdheartman. Sorry that you have to be here, but you've come to the right place for support. My son's aorta dissected and he has permanent heart damage now so I am biased on the side of getting it done now.

I would meet with your prospective surgeons and pose the why wait questions. Maybe they think that you will not need surgery for another 5+ years. If that is the case, medical advances may change the way the surgery is performed.

I don't have any suggestions for your other questions.

Please keep us posted.

 

[Ross]

I'm a ruptured aneursym survivor and mine never made it to the magical 5.0! Mine blew at around 4.7. Once your at 5.0, I see no reason to wait. They seem to like to try to push it to 6.0 and I think that's totally unnecessary and bordering on downright dangerous.

 

[MrP]

Regarding timing of surgery, this article from Yale may prove useful.

http://72.14.203.104/search?q=cache...+valve+aneurysm+yale&hl=en&gl=us&ct=clnk&cd=9


All my best,
MrP

 

[skeptic49]

Welcone to VR! Really good post from Reddog...just about says it all.

Good luck and best wishes,

Jim

 

[Maryka]

Hi and welcome! I cannot respond to many of your questions, but have some info on some of them. First, I had my BAV and aneurysm surgery at Johns Hopkins with Dr. Duke Cameron. (He was promoted to Head of Cardiology the day after he completed my surgery. Don't you think he owes me a letter of thanks for that?) Hopkins folks know a lot about valve-sparing surgery, I believe. If you live close to Baltimore, I recommend Hopkins.

Aneurysms can stabilize and even shrink. My aneurysm (approx. 4.2 cm.) was discovered in a routine chest X-ray, believe it or not. I immediately went on beta blockers and quit exercising, etc. Although different CT scans and MRIs showed slight variation in size, there is a margin of error in those tests. (Echocardiograms never picked up my aneurysm--up near but not quite at the arch.) My aneurysm literally stayed the same size. (Meanwhile my "little aortic valve murmer" totally deteriorated.)

I know a Marfan woman who went on Cozaar before it was realized it could reduce aneurysms. Her aneurysm shrank. Hal Dietz (Hopkins geneticist) and others realized a few years back that Cozaar can reverse some genetic problems because some genetic problems get worse because the human body actually attacks the genetic defect, making it worse. Marfan, and some other problems (like some forms of Muscular Dystrophy) respond positively to Cozaar.

My aneurysm did not respond to Cozaar and my bicuspid aortic valve got worse and worse. (I probably am not Marfan, as I was diagnosed years ago.)

Around Maryland, aneurysms are repaired at 5 CM. I am no doctor or expert, but your aneurysms sound more than big enough to repair. As for valve sparing--do you really want to face more surgeries in the future? I chose to have a mechanical valve and have my 4.2 cm aneurysm repaired at the same time. I hope that I will never again have to face OHS in my lifetime. (But, as Ross will tell you, there are no guarantees as far these surgeries go.)

God be with you as you make this big decision! I know several people who were operated on by Craig Miller. He is a great surgeon. (Dr. Liang at Stanford was the first doctor to realize that my real problem was probably not Marfan, but a congenital bicuspid aortic valve. Bless his heart!).

 

[COLLEEN S]

Duke Cameron was my surgeon. He is one the best with valve sparing. He looked at mine while in my chest and immediately knew, due to the wide open, shredded valve (my cardiologists words), that I was not a candidate for valve sparing. He is a great man, monitors patients FROM HIS HOME, has taken calls from me on his cell phone, has answered every question I have had. Not a showy, know it all...very low key and humble despite his talents.