An uncomfortable case of the patient directing the doctor...

[PJmomrunner]

A quick synopsis: I'm 42, female, purely regurgitant moderately insufficient BAV, 4.3cm to 4.5cm ascending aortic aneurysm, average BP 119/79, on Toprol 25mg x 2/day, mildly symptomatic at most.

I recently had a consultation with a CT surgeon (who shall remain nameless on this forum, 'cuz it seems right not to name him) who is a specialist in bicuspid aortic disease. During our consultation I related to the surgeon some of the "information" my cardiologist has been slinging my way: "Your dilated aorta is a compensatory mechanism--your body's way of getting you more blood." "It's not a symptom of disease like Marfan's or an artherosclerotic aorta or a syphillitic aorta. It's like opening another lane on the freeway." The surgeon encouraged me to "get my (clueless) cardiologist on board" and said that most cardiologists are not up to speed on this stuff. His (wonderfully helpful) staff sent me a couple of papers (Fedak's 34-year-old pilot with BAV paper, and a Feb. '05 paper by Catherine Otto et al).

Today I mailed my cardiologist a letter explaining to him that I had a telephone consultation with this surgeon, who recommended to me that my systolic blood pressure be kept between 105 and 110 during normal activity. He said that his research shows that maintaining the BP in this range results in systolic BP while exercising not exceeding 135. The surgeon noted that most of his patients are on both a beta blocker (which I am on) and an Ace inhibitor (which I am not on...yet) to achieve this range. In my letter I relayed this info and requested my cardiologist's assistance in achieving that range and made an appointment for July 5 to discuss the matter. I also sent along with my letter copies of the aforementioned papers and a print out of the Cedars Sinai website's section on BAD.

While I'm proud of myself for taking charge (many of you have been helpful in encouraging each other to do that), I feel quite uncomfortable with directing my own care and educating my cardiologist this way. I anticipate that my July 5 appointment will be quite uncomfortable, and yet I can't accept the idea of suboptimal care. I live in a small town and feel confident that I'd have to get any of the local cardiologists "on board," so switching cardiologists would probably gain me nothing. Besides, I believe he sincerely cares, which is not everything, but it helps.

I know I'm not the only one who has ever gone down this road.... Those of you who have been here, how did you handle it? I could use your input/support/illucidation before I go to that appointment.

As always, thanks for "listening."

P. J.

 

[Nancy]

Well, Joe had to find a cardiologist who was a specialist in advanced cardiology and really understands the complexity of his problems. We are fortunate that there is a large teaching hospital not too far away. But still, Joe has to have another 6-7 specialists on his roster of physicians. And his main doctor is his Internist who is excellent.

We have found that they do not necessarily communicate with each other, and the only ones who understand the WHOLE picture are Joe and I.

I feel strongly that it is a partnership with us and all of his physicians and we work in tandem. There's a tremendous amount of give and take in all the interactions. I don't think it's necessarily a bad thing that you have some ideas of your own, and your cardiologist welcomes them. I think it's a good thing. It's a bad thing if you have some good ideas and are right about some things but the cardiologist or other doctor ignores you.

And we don't mind if we come up with an idea that is not so good, and it is pointed out. It gives a good point for discussion which might come up with a wonderful solution.

Some things are just too complex for one person to know. Working together is good, as long as the people involved have open and curious minds, and respect each other.

 

[char2mar]

P.J

Seems you and I are reading from the same page. I just had a talk with my internist/cardiologist about BAVD and had to ask if he knew anything about the disease becasue he was trying to tell me that I had nothing to worry about and that I wasn't going to drop dead anytime soon. He seems to feel that my dilated aorta and moderate regurgitation wouldn't get any worse and that perhaps the chest pain I am experiencing was only anxioty. (LOL) So I just said "Do you know anything at all about BAVD?" He looked shocked that I should ask such a question...but sat there glummly as I listed off all of my symptoms and test results and commonalities I have to this disease. I suggested that perhaps he look it up on the net like I had too because it was his job to provide me with the best possible treatment and provide me with the right information so that I don't get stressed out or worry too much about it. Then I told him that the other internist I was seeing basically felt the say way as he did but realized that I should be sent to someone who may be able to help me. To make a long story short he basically told me that he was unable to help me too and that he wished me luck and then he patted me on the head and said he wished he could make all my worries go away. I felt like kicking him at that point. So I thanked him for his service and walked out of the office. Thankfully I won't have to see him again! He seemed to be a nice guy and generally caring but I don't think he liked having one of his patients question his knowledge. So now I will have to wait until July 8th to go see yet another doctor. This one works out of the Adult Congenital Heart Clinic. Hopefully he will know more than the other doctors I have seen. All I want is a proactive doctor who explains things to me, listens too what I have to say and sets up some kind of game plan to keep me fairly healthy and well. What I don't want is to have a crisis situation and have all hell break loose at the expence of my health and general well being. I have seen all too well what happens in a crisis situation. Besides I shouldn't have to walk around for months feeling like crap. What do you think?

Char

 

[PJmomrunner]

Thanks for the replies. Nancy, you are fortunate to have a teaching hospital nearby. I do feel that when I've spoken with the folks at the two large teaching hospitals I have visited I am more comfortable with asking questions. Maybe they are accustomed to fielding questions and confident enough in their own abilities to not feel threatened by inquiries. That's not exactly the feeling I get locally, but he was receptive enough to Marfan Syndrome info I brought on my first appt., so maybe he'll rise to the occasion again. It's too soon in my process to get weary, but I wish he just knew all about bicuspid aortic valve disease.

Char, you seem orders of magnitude more frustrated than I feel. I can't imagine how it must feel to have the symptoms you describe and be, more or less, blown off by your doctors. I do feel like I was more symptomatic when I was spending a lot of time on this website and a lot of time on PubMed doing research and a lot of time just plain worrying and I suppose one could chalk some of that up to anxiety. The thing is, that's not anxiety about an asteroid hitting earth and killing us all, it's a pretty rational sort of anxiety that, in my case, is greatly diminished by my gaining understanding of the situation and my trusting that it is under as much control as it can be.

I can't fully trust that it's under control when my cardiologist doesn't seem to grasp the situation, and it sounds like you can't either when you have symptoms nobody seems to understand, or even wants to try to understand. In my case I can try to help the cardio help me (we'll see if that helps or hinders) by providing info. In your case, all you can do is blow off steam with us here and let the docs see the calm Char who patiently (pun intended) reiterates and inquires and suggests until you've gathered enough info to have a plan you can live with.

So, we'll all plug on together!

Thanks again.

P. J.

 

[BillCobit]

He seems to feel that my dilated aorta and moderate regurgitation wouldn't get any worse and that perhaps the chest pain I am experiencing was only anxioty. (LOL) So I just said "Do you know anything at all about BAVD?" He looked shocked that I should ask such a question...but sat there glummly as I listed off all of my symptoms and test results and commonalities I have to this disease.

Good for you, Char! The guy certainly deserved that, especially after dismissing chest pain as probable anxiety. Hope you've found a better doc since then.

It's a pretty rational sort of anxiety that, in my case, is greatly diminished by my gaining understanding of the situation and my trusting that it is under as much control as it can be.
P. J.

PJ, Keep reading and trust your instincts! It would be comforting (but naïve) to trust that any cardiologist is up to speed on every condition. If you feel like your care is "self-directed" or materially inconsistent with what you are reading, you have the wrong guy/lady for a cardio doc. I'm not trying to stoke anxiety, but you've read enough to know that it's important to get treatment of valve/aorta problems right the first time; don't leave yourself in the wrong hands. It can be tiring and inconvenient, but find somebody who is knowledgeable and who takes interest in you as a patient, and is comfortable working with an "informed consumer."

 

[Mary]

P.J.,
It seems like you will need to look for a cardiologist in a larger city if you feel you are directing your own health care. Many people on the Forum have encountered the same problem, and many have chosen to look elsewhere for care.

However, if you've seen a surgeon (and I thought you had), then you can always fall back on his judgment. It's pretty time consuming to get a topnotch cardio and surgeon, but that's a choice you'll have to make.

 

[PJmomrunner]

Thanks for the validation Bill. I do feel like my overall care, in the hands of the surgeon, is right where it should be. The suggestion for lowering my BP came in through a side door, so to speak, but from a highly qualified surgeon as well. Lowering my BP makes sense to me, I guess I'm just being a wuss wishing the idea had come from my cardio so I don't have to be the "know it all" asking to do it.

 

[McCln]

It is bad when the surgeon runs down a fellow doctor, a cardio no less. There is times when a cardio and surgeon does not communicate well at all. They need to commincate to help you get the right kind of care. I wish you luck when you do go back to see the cardio. You see, the surgeon send his report to your cardio already, so all you have to do is show up and discuss the situation of the war of the minds. Majority of cardio's are always up to speed on the latest treaments and conditions, they have to, specialists keep going back to school to get caught up and the reading they do from the Journal of Medicine. Good luck and hope they reach an understanding soon. Do not stress over this, it happens a lot. Doctors are not always friends to each other.

 

[tobagotwo]

It's hard to change the relationship between yourself and doctors. You've grown up to trust them with you physical well-being, and to leave their world to them.

But those days seem gone when you have this type of thing to deal with. You find that you either need a doctor who's willing to listen to you, and wants to try to keep up with (or ahead of) you, or one who already is ahead of you.

I agree that you are doing the right thing. Many of us wound up cardio-hopping when we started looking for real answers, so you are not alone, by a shot. You need to be comfortable that you're not the only one who knows what's going on inside your chest.

I'm also educating my cardiologist about my little niche of the medical world, I find. This is okay with me, because I like him in general, and I believe he is basically a good match for me. Of course he is an excellent cardiologist, and I'm only up to date on my very limited portion of it, so it's no discredit to him, his heart knowledge, or his larger practice. He's an outstanding interventional cardiologist (does stents and catheterizations).

I've just had my procedure about a year ago, so I don't have the critical requirement that you do going into it. I hope you do find someone who will fill the bill for your needs soon.

Best wishes,

 

[Debrinha GT]

Here's what I did...

Here's what I did...

I've had the same cardio for eleven years now, and although he answers most of my questions, he doesn't go into much detail. Last year I just felt I had to go and see my surgeon because I really believed he was the right person to clear up some of my doubts since he was the one who operated on me the last time. I have private health care and here in Brazil you can go to a doctor any time you want to, I mean, I didn't need my cardio's permission. Dr. Costa was great and we discussed a lot of things. One of the things I like about him is that he doesn't beat about the bush and I was happy with his straight answers. As wel as that, he doesn't treat the patient as if he was an idiot. When I went back to see the cardio a couple of months later, I told him about it but he was already aware of it. There were no hard feelings and since he knows what I'm like, he knew that I wasn't doubting his word. What I've learned after having had three mitral valve rplacements is that you'd better be safe than sorry.
Débora

 

[PJmomrunner]

Thanks for sharing your experience, Debora. You have the rather dubious distinction of being a very seasoned veteran!

Thanks for your support as well, Caroline. I guess doctors are human afterall.

Bob, as usual you are right on point. You remind me that I actually have been in this position before--with childbirth. I was the "don't-intervene-in-any-way, very assertive, here-read-my-birthplan, earth mama." (The biggest difference is that with childbirth the my whole premise was that it was something normal and natural and something that would pretty much happen on its own. The prospect of managing a dissection is not normal in any way, I guess it's kind of natural in my case, but if it just happens I'm in deep trouble!) But you're right, it IS about changing the doctor-patient relationship concept and that's what makes it uncomfortable. It is nice to know that I've got good company.

 

[karlaosh]

Hi P.J.

Having spoken with Zehr at Mayo a number of times now, my perception is that each leading heart center is somewhat of an island unto itself.

I asked Zehr about the hemiarch procedure that Raissi recommends for bicuspids. He a) seemed never to have heard of Raissi (how can that be???) and b) said that risking a hemiarch was crazy because Mayo knows that 10% of their circulatory arrest patients suffer a stroke post-surgery and that that percentage is much higher than the long-term complication rate they have on BAVD patients who have their valves and ascending aortas replaced but no arch work done.

I believe Mayo's numbers and I trust Zehr's reliance on those numbers. What doesn't make sense to me is how come Mayo isn't looking at Cedars-Sinai's numbers and vice versa, and how come someone like Kenton Zehr isn't closely following Sharo Raissi's work and vice versa. (Zehr is highly published on aortic matters in the medical journals.)

From what I could tell, Zehr still abides by the "asending aortic aneurysm as a result of increased pressure from the valve" vs. the connective tissue theory that Raissi champions. And, interestly, my path report doesn't say anything about connective tissue disease (though I only have a summary path statement on my discharge papers; I'm going to get the full path report to see what it really says).

The question is, how do we get these leading aortic folks to start talking to one another?

And in the meantime, who do we believe?

I do think you're right to follow Raissi's recommendations on the meds and force your local cardiologist to get on board. After all, a more conservative medical approach can only help (right?).

Oy vay.

Karla

 

[PJmomrunner]

Hi Karla:

It's good to see you posting! I trust all is well and getting better?

My guess is Raissi's name is familiar to all of us because we're a bunch of websurfing forum rats and Cedars-Sinai's website is a high-profile hit! It's also the only hospital website I've seen that's bold enough to put a lot of information out there for all of us to see. That Cedars is in perhaps the most litigious region of the country and that they have all that info on their website means to me that they can strongly support everything they state. Their website is a tremendous service to we patients, but is it peer-reviewed the way journal publications are or is it only lawyer-reviewed? I think we would all benefit if the Zehrs and Raissis and Deebs of the world would get together and come up with a website.

If you look on VR.com's reference forum you will see a FEb. '05 paper I recently posted that was peer-reviewed and printed in Circulation. It's sort of an editorial "what we know about bicuspid valves" and it very much supports Raissi's/Cedar's statements.

Personally I don't see how one can argue with studies that report larger than average aortic diameters associated with bicuspid valves consistently in infants through the very old and studies that show that the aorta continues to dilate after the valve has been replaced and studies that examine bicuspid aortic tissue and find an abnormal cellular structure. BUT whether all that constitutes a systemic tissue disorder...whether that precludes that turbulent bloodstream's having a destructive effect...I'm not convinced.

I don't know about the hemiarch thing. I'm going to hope to see some good dialogue on that on PubMed. I don't understand what it is exactly, but I think both Raissi and Deeb favor doing one on me. It's not clear to me whether either or both favors it for all BAVD people.

Thanks for weighing in on the blood pressure issue and everything else too. I sure do enjoy the dialogue, even if the surgeons don't seem to do the same!

Take care Karla.

P. J.

Hi P.J.

Having spoken with Zehr at Mayo a number of times now, my perception is that each leading heart center is somewhat of an island unto itself.

I asked Zehr about the hemiarch procedure that Raissi recommends for bicuspids. He a) seemed never to have heard of Raissi (how can that be???) and b) said that risking a hemiarch was crazy because Mayo knows that 10% of their circulatory arrest patients suffer a stroke post-surgery and that that percentage is much higher than the long-term complication rate they have on BAVD patients who have their valves and ascending aortas replaced but no arch work done.

I believe Mayo's numbers and I trust Zehr's reliance on those numbers. What doesn't make sense to me is how come Mayo isn't looking at Cedars-Sinai's numbers and vice versa, and how come someone like Kenton Zehr isn't closely following Sharo Raissi's work and vice versa. (Zehr is highly published on aortic matters in the medical journals.)

From what I could tell, Zehr still abides by the "asending aortic aneurysm as a result of increased pressure from the valve" vs. the connective tissue theory that Raissi champions. And, interestly, my path report doesn't say anything about connective tissue disease (though I only have a summary path statement on my discharge papers; I'm going to get the full path report to see what it really says).

The question is, how do we get these leading aortic folks to start talking to one another?

And in the meantime, who do we believe?

I do think you're right to follow Raissi's recommendations on the meds and force your local cardiologist to get on board. After all, a more conservative medical approach can only help (right?).

Oy vay.

Karla

 

[jax3172]

BAV and arterial issues

BAV and arterial issues

Karla,

I found the following article that suggests that there is a correlation between less than perfect arterial tissue, in general, and BAV:

http://stroke.ahajournals.org/cgi/content/full/26/10/1935#R43

I quote: "...The familial occurrence of spontaneous arterial dissections and BAV suggests a common developmental defect. The aortic valvular cusps and the arterial media of the aortic arch and its branches are derived from neural crest cells, suggesting that a neural crest defect may be the underlying abnormality in these families."

Very interesting!

 

[Arlyss]

Regarding Surgical Approach to the Aorta

Regarding Surgical Approach to the Aorta

Surgery involving the aortic arch, including a "hemi-arch", is only performed when it is needed. "Hemi-arch" refers to those situations where the tissue on the "bottom" or underside of the arch is found to be thin and abnormal, and when that is the case, it can be removed along with the ascending aorta.

My husband has a small part of his Dacron graft extending into the underside of his arch. Some people might need even more Dacron than he has under his arch, and Dacron might replace the entire bottom half of their arch. Others might not need anything at all done to their arch.

Circulatory arrest is needed when working on the arch. Circulatory arrest is also needed to remove the entire ascending aorta, even when the arch is not included in the surgery. In removing the entire ascending aorta, a clamp is not used and the aorta is "open" while the Dacron graft is being attached. Circulation is stopped while this is done.

If I had already had surgery on my ascending aorta, I would want to know if it was all removed or if the portion near the arch was still there. If I still have some part of my ascending aorta, I would want to be monitored in case I should develop an aneurysm there later. Even though my husband has had his entire ascending aorta replaced, he does have follow up MRIs which evaluate his valves and heart chambers (some centers now offer cardiac MRIs) as well as his aorta. His aorta continues to look great four years post op!

Regarding the debate about the effect of abnormal blood flow through the bicuspid valve causing the aorta to enlarge, I can tell you that my husband's aorta grew to aneurysmal size after his bicuspid aortic valve was replaced, when he had much better blood flow through his mechanical valve. Actually, since he had stenosis, his aorta saw a much greater volume of blood flow after his valve was replaced. There is a study from Milan, Italy, that followed those with bicuspid aortic valves after their valves were replaced.
Here it is
http://www.ncbi.nlm.nih.gov/entrez/...d&dopt=Abstract&list_uids=12440663&query_hl=1

It might be reassuring to know there is a surgical forum that focuses exclusively on the aorta in the chest. It is held every two years in New York, and surgeons from around the world attend.

I hope some of this helps!

Best Wishes,
Arlyss

 

[PJmomrunner]

Jax: Thanks for posting the link to that paper. It certainly supports the idea that BAV people are prone to all kinds of aneurysms. It also makes me wonder about whether hernias have some sort of tissue-based relationship to BAV as well.

Arlyss: Thanks for explaining the hemiarch procedure. I wonder, do they replace only the bottom of the arch for expediency's sake? (e. g. Replacing the entire arch and "buttoning" in the head vessels would take too long while patient is under hypothermic arrest?)

I had my "lower blood pressure, please" appointment with my cardiologist today. He explained that beta blockers are the BP drug of choice for people with aneurysms because they work by "blunting" the time (milliseconds) it takes the BP to rise or fall--effectively eliminating immediate BP spikes. (It will still rise some, but not as rapidly.) He expressed concern that having one's BP too low could cause grogginess or passing out, etc. He did consent to giving it a try, although he felt it was fine where it was, and he increased my Toprol XL dose to 75 mg/day. I am to continue monitoring my BP.

 

[karlaosh]

FYI: My ascending aorta was replaced and I wasn't under circulatory arrest.

 

[Arlyss]

Just A Little More Clarification

Just A Little More Clarification

The entire arch, just like any other section of the aorta, is replaced when the arch tissue is clearly abnormal, stretched, and thin everywhere. It seems fairly common that just the underside of the arch is what gets in trouble.

It is possible to have most but not all of the ascending aorta replaced, and then circulatory arrest would not be used because the small piece of aorta that is left can be clamped. To take it all, right up to the arch, circulatory arrest is used. Here is a link to an interesting panel discussion about ascending aortic surgery, and the views of different surgeons.

http://ats.ctsnetjournals.org/cgi/content/full/74/5/S1792

This panel discussion covers several areas, including the possibility of cross clamp injury to thin aortas. It is also mentioned that taking an extra 15 to 30 minutes to remove the distal (next to the arch) ascending aorta may save 10% to 20% of people from having another operation due to a problem with this small piece (2-3 cm) of ascending aorta that was left behind. Using circulatory arrest makes it possible to replace the 2-3 cm of the ascending aorta right next to the arch. When done this way, no ascending aortic tissue that was clamped is left behind.

If I know that the small piece of my ascending aorta right next to my arch, which was clamped during surgery, is still there, I would just want to be monitored, in case I belong to the 10-20% of people that have problems there later.

Reading Nancy's comments earlier in this thread, I also agree that it is best when there is a partnership between medical professionals and the patient/family. I hope everyone can find/develop that kind of relationship.

Arlyss