28 yrs old with BAV & Severe AR

[Deidra]

Hey everyone!

I wanted to thank you for being on this forum and express my gratitude for its existence. Reading through everyone's experiences & thoughts here has been very informative and comforting.

I'm 28 years old, lightly-moderately active, not/minimally symptomatic with a BAV, severe AR and a mildly dilated aortic root (4.2cm, I'm 6'2"). It's been under surveillance since I was a teenager, skipped a few years, found the dilation and moderate-severe regurgitation when I was 24. Root has stayed the same, regurgitation was up to severe on last check and EF was 54%, from an average of 58% over the past few years.

I met my new cardiologist at OHSU in Oregon on Monday and she was very kind. We've scheduled an echo and a cardiac CT and if things look any worse than the prior echo we'll probably be moving forward with surgery, most likely an Aortic Valve Replacement with potential Aortic Root Replacement/Remodeling. There is an outside chance of a repair, but I may have to seek a second opinion from a center with lots of BAV repair experience on that. At the end of the day, I recognize that repair is only a chance and I would be thrilled to get a mechanical valve and hopefully call it a one-and-done. I'm diligent, very invested in my health, and have a science background, so I am eager to get to INR self-managing if/when I end up getting a mechanical valve. (Thank you to pellicle and everyone else here who has provided invaluable info on this particular topic).

I was warned that my (likely) surgeons are very eager about the Ross procedure, but my cardiologist was very supportive of my self-advocacy and choice of valve. I don't see the benefit of turning one sick valve into two and doing a more complicated surgery just to POTENTIALLY push any reops 15-20 years down the line in an ideal circumstance, which is much less likely given that from what I've read having severe AR and a dilated annulus predisposes Ross procedures to early reop. If I can get a chance to do a one-and-done, I'm going to take it. The newer data about bleeding/TE risks with optimal INR self-management for mechanical valves is very compelling and reassuring.

I feel good about where I'm at and my chances at having a long and happy life. I recognize that I'm in a very good position in terms of pre-operative risk, being a healthy 28 year old with no significant health issues other than the valve/aortopathy. I've had a lot of time to think over everything and emotionally and mentally prepare myself. Sure, I'm anxious, but I'm also excited to get this over with and start my recovery, and so grateful that this treatment option even exists at all, and that I have access to it. I know my life has changed and will never be "back to normal", but my life hasn't exactly been normal up to now so I'm not broken up about that.

Thanks to everyone again for reading and sharing your thoughts on this forum

 

[Chuck C]

Welcome to the forum Deidra!

I'm glad that you found us. I found this forum a few months before I needed surgery for my BAV and it was a tremendous help to me. It sounds like you are making full use of this resource as well.

I was warned that my (likely) surgeons are very eager about the Ross procedure, but my cardiologist was very supportive of my self-advocacy and choice of valve. I don't see the benefit of turning one sick valve into two and doing a more complicated surgery just to POTENTIALLY push any reops 15-20 years down the line in an ideal circumstance,

In my view, your thinking about this is spot on. The guidelines do not call for a Ross procedure for a young patient and there are surgeons who are eager to do them. At 28 you should be thinking not just 20 years ahead, but 50+ years ahead. From all appearances, this seems to be what you are doing. For a 28 year old patient, the Ross would guarantee several future procedures.

At the end of the day, I recognize that repair is only a chance and I would be thrilled to get a mechanical valve and hopefully call it a one-and-done.

I hope that a repair is possible, but you are spot on that mechanical is your best bet to be one and done and it is exactly what the guidelines call for in the event that a 28 year old requires aortic valve replacement.

I'm diligent, very invested in my health, and have a science background, so I am eager to get to INR self-managing if/when I end up getting a mechanical valve.

Excellent. Self testing is the optimal way to go for most people. As I expect you have probably read in your due diligence, self managing can drop the risk of having events by about 50%, close to that of the general population.

I feel good about where I'm at and my chances at having a long and happy life. I recognize that I'm in a very good position in terms of pre-operative risk, being a healthy 28 year old with no significant health issues other than the valve/aortopathy.

You have a great attitude. And you are correct that you should expect a long and happy life.

My only suggestion would be to make sure that you don't wait too long for surgery. Since you are probably getting a mechanical valve and this will likely be the only procedure you will ever need, probably the only risk would be waiting too long to the point that your heart does not remodel completely after surgery, which could lead to some issues later in life.

My situation was BAV with aortic stenosis, and I am not as knowledgeable about regurgitation, but I believe that the indications for surgery are similar. When my stenosis became severe, I was informed by my cardiologist that I could get surgery now or wait for symptoms. Since I had decided on a mechanical valve, it made no sense to wait for symptoms, as it was likely to be one and done. When I made my list of pros and cons, as to getting surgery now or waiting for symptoms, I really could not come up with any benefit to waiting. My surgeon agreed with me and we scheduled my procedure about 3 weeks from our last consult. He pointed out that there was significant risk to waiting and that the first symptom might be sudden death. The medical literature was very clear that long term outcomes were better for severe AS patients if surgery was done before symptoms. It seemed that waiting for symptoms would just be kicking the can down the road a little, for what I hoped would be a one and done procedure. This literature was for AS and not AR, so I can't speak to any medical literature regarding AR with respect to surgery now or wait.

It sounds like you have the right attitude and have a good head on your shoulders. Your team is on top of your situation and they respect your self-advocacy. This is all good news.

Please keep us posted on your journey.

 

[Deidra]

Hey Chuck, thanks so much for writing back and sharing your thoughts & experience! I hope you are doing well. It's really nice to talk to other people who have experience with this. I don't know anyone in my life who has had a valve replacement or even OHS in general.

My only suggestion would be to make sure that you don't wait too long for surgery. Since you are probably getting a mechanical valve and this will likely be the only procedure you will ever need, probably the only risk would be waiting too long to the point that your heart does not remodel completely after surgery, which could lead to some issues later in life.

100% agreed and I think this is really good advice. Based on what I've read about AR, it's a very similar situation in that your heart can only compensate so much before there starts to be irreversible damage.

I don't want to put the surgery off and honestly I'm eager to get it done with, I don't see the point in waiting. I have an chest echo next Wednesday the 31st and a follow up with my cardiologist in May, so that will probably be when I know for sure

 

[3mm]

my (likely) surgeons are very eager about the Ross procedure

Perhaps consider other hospitals for your surgery. I went 3 hours north of my home to Mayo Clinic to ensure I had an entire team who were highly experienced in valve repair and replacement.

Because this may be important for you, I will risk being politically incorrect. If you are female and want to someday have a child, please consider how Warfarin interacts with pregnancy. Your decision, your life, etc. You do NOT owe me any response on this.

 

[Deidra]

Perhaps consider other hospitals for your surgery. I went 3 hours north of my home to Mayo Clinic to ensure I had an entire team who were highly experienced in valve repair and replacement.

Hey Mark! Yeah I think this is a good point, thank you. I feel pretty confident about the team at OHSU so far, but I haven't met the surgeons yet of course. UW in Seattle has a good valve team from what I can tell, and Cedars-Sinai and UCLA are a couple days away by car as well, so I have some options.

I did some looking on the STS Public Reporting database and all four hospitals have similar stats for valve replacements, but obviously that's not the be-all end-all.

If anyone has experience with valve replacements in the PNW please let me know!

Edit: This is mostly unrelated but a fun fact I just learned. Apparently Dr. Albert Starr was director of cardiac surgery at OHSU until the 1990s, and the first Edwards-Starr valve was implanted into a young woman at OHSU.

 

[Chuck C]

Cedars-Sinai and UCLA are a couple days away by car as well,

I had surgical consults at both Cedar Sinai and UCLA and was very impressed with both. Although, the head surgeon at Cedar Sinai seemed a little too eager to operate, suggesting that I get SAVR immediately, even though I was only moderate AS, and asymptomatic. 20 months later, I ultimately ended up getting my procedure done at UCLA once my stenosis became severe. I give them high marks across the board for valve surgery.

It is remarkable how opinions can vary, in terms of when to operate. My local cardiologist believed I was somewhere between 5 and 20 years away from needing surgery. Right after that I got the surgical consult at Cedar Sinai, and using the exact same echo data, was told that he'd like to schedule me for the next week for SAVR.

 

[Teapotimus]

OHSU in Oregon

but I may have to seek a second opinion

Greetings

I was a patient at OHSU until September of 2023, had appointments with their entire team, Drs Aldweib, Broberg, Khan, and most recently McGrath. Now I'm a patient at Providence, which took some effort as they have a policy to refer all congenital patients to OHSU.

My experience with OHSU was not great, very kind but a lot of unnecessary stress on me. On two occasions I was told my LV was dilated (once mildly the other severely) but upon reinvestigation was found to be completely in the normal range (over measured by nearly 1cm).

I was also offered annual imaging instead of biannual due to improvement in my condition they can't explain.. Even though the previous appointment they figured I was 6 months from surgery.

As for the Ross I've had the same debate as you, though I believe both to be good options provided the Ross is done by someone who is truly an expert at it, of which there are very few in the country. I wouldn't touch the Ross at OHSU, they have very poor outcomes with it in both the STS database and a study Dr. McGrath is an author on analyzing the Ross from 2005 to 2019 at OHSU.

That being said there was a recent single center publication from the UK of a Ross cohort entering the third postoperative decade with impressive numbers. I believe it's based on a RCT of which there is only one other involving the Ross procedure, I imagine these studies may influence the guidelines in the future.

 

[Lucker]

Hello and welcome. Your disregarding of Ross procedure seems to me quite prudent, considering your height, gender and young age, that may presuppose some variation of systemic connective tissue disorder or even Marfan. I am tall and have AR too, and talked to a geneticist who thought I have some traits of connective tissue disorder.

 

[Deidra]

Greetings

I was a patient at OHSU until September of 2023, had appointments with their entire team, Drs Aldweib, Broberg, Khan, and most recently McGrath. Now I'm a patient at Providence, which took some effort as they have a policy to refer all congenital patients to OHSU.

My experience with OHSU was not great, very kind but a lot of unnecessary stress on me. On two occasions I was told my LV was dilated (once mildly the other severely) but upon reinvestigation was found to be completely in the normal range (over measured by nearly 1cm).

I was also offered annual imaging instead of biannual due to improvement in my condition they can't explain.. Even though the previous appointment they figured I was 6 months from surgery.

As for the Ross I've had the same debate as you, though I believe both to be good options provided the Ross is done by someone who is truly an expert at it, of which there are very few in the country. I wouldn't touch the Ross at OHSU, they have very poor outcomes with it in both the STS database and a study Dr. McGrath is an author on analyzing the Ross from 2005 to 2019 at OHSU.

Hey! Thanks so much for sharing your experience.

That's very interesting. Dr. McGrath is my new cardiologist and I just saw her Monday. She agreed with me that mechanical is the way to go if a durable repair isn't feasible. I agree about the high technical requirements for the Ross. Based on the data I've seen, I'm not a great candidate for it due to the AR and dilated root, as it seems to do better in people with stenosis and normal root sizes. I'd rather not take the gamble and get something time-tested like the St. Jude.

My story is kinda complicated and things definitely haven't been perfect at OHSU for me so far:

I was at Legacy Mt. Hood Medical Center before but I switched to OHSU. When I first got an TTE done at Legacy, they graded my regurgitation as "trace" and the cardiologist said to come back in 3 years. I was very suspicious about this because it had preivously been graded as moderate-severe. So I came back a year later, and my TTE was done by a very inexperienced student that time. It showed severe regurgitation and a lower EF as I mentioned in my post. I got kinda fed up and heard good things about OHSU. When I got there I had an appointment with Dr. Aldweib who was kind and helpful (also 50 minutes late due to a hospital emergency, but that's understandable), said he would schedule me for a cMRI to see what's going on. However, I got a call a few months later that he was no longer seeing Adult CHD patients and I'd have to see Dr. McGrath instead. Never heard about that cMRI even though I called multiple times. So now I've seen Dr. McGrath who scheduled me for an echo next week and a cardiac CT scan soon. Once I get the referral to the surgeons I'll talk to them and see about second opinions.

How has your experience been so far at Providence?

 

[Deidra]

Hello and welcome. Your disregarding of Ross procedure seems to me quite prudent, considering your height, gender and young age, that may presuppose some variation of systemic connective tissue disorder or even Marfan. I am tall and have AR too, and talked to a geneticist who thought I have some traits of connective tissue disorder.

Hey! Thank you. Yeah my previous cardiologist had a similar concern, so I had a genetic panel for Aortopathy including Marfan and EDS done a year ago that came back all negative.

However, based on my understanding, these panels only test for the commonly known and proven genetic anomalies that cause well-known syndromes like Marfans. As far as I'm aware, the genetic causes of BAV and related aortic disease have not been fully nailed down. So yeah I'm inclined to believe there's a good chance I have some kind of underlying connective tissue disease related to the BAV, even if it's localized to the Aortic Valve & Root, despite the negative panel result. Which would definitely be a reason to not get the Ross.

 

[Deidra]

I had surgical consults at both Cedar Sinai and UCLA and was very impressed with both. Although, the head surgeon at Cedar Sinai seemed a little too eager to operate, suggesting that I get SAVR immediately, even though I was only moderate AS, and asymptomatic. 20 months later, I ultimately ended up getting my procedure done at UCLA once my stenosis became severe. I give them high marks across the board for valve surgery.

It is remarkable how opinions can vary, in terms of when to operate. My local cardiologist believed I was somewhere between 5 and 20 years away from needing surgery. Right after that I got the surgical consult at Cedar Sinai, and using the exact same echo data, was told that he'd like to schedule me for the next week for SAVR.

Thanks for the info! That's really interesting yeah. I wonder what that sugeon at Cedars-Sinai was seeing/thinking. I guess it makes sense that surgeons just want to do surgery hah.

My cardiologist at Yale-New Haven in CT at 24 looked at me with moderate-severe regurgitation and a 4.2cm root and said I'd need surgery in 5-10 years. So far that's looking like a pretty accurate prognostication.

 

[Teapotimus]

When I got there I had an appointment with Dr. Aldweib who was kind and helpful

Dr. Aldweib was my primary back in 2021, but when it came time for my second appointment he was gone for a few months which led me to see Dr. Broberg. Then Dr. Aldweib came back and like you I was told when trying to schedule my next appointment he was no longer seeing congenital patients, Though he's still listed on their website under the congenital cardiology team... This led me to seeing Dr. McGrath last September.

Dr. Aldweib also confusingly suggested I go from biannual imaging to annual imaging the last appointment I had with him. This was after telling me the whole team thought I would need surgery within 6 months 3 years ago.

She agreed with me that mechanical is the way to go if a durable repair isn't feasible.

I found Dr. McGrath to be very kind and if I decided to go with a mechanical valve I appreciated that she's very pro self INR management. What pushed me over the edge with OHSU though was the fact that she had 0 knowledge of my medical history prior to my appointment with her, and the same situation had happened with Dr. Broberg earlier in 2022.

My appointment with her was following an Echocardiogram which found my LV to be dilated (again...) and she started the appointment trying to discuss surgery (6 months after Dr. Aldweib suggested I only come in once a year due to my AS and AR improving fairly dramatically). When I pointed out that I had this exact situation occur (LV dilation that wasn't accurate) at OHSU a year or so prior she scrambled to look back at my notes and shifted to suggesting another cMRI to clear up the confusion (exactly what happened following an Echocardiogram with Dr. Aldweib where Dr. Khan reported severe LV dilatation).

How has your experience been so far at Providence?

It was a challenge to get in, if you mention the word 'congenital' to their schedulers they'll tell you they refer all congenital patients to OHSU. However I've had an excellent time so far though it's only been two appointments.

Currently I'm seeing Dr. Moon at the Newberg location, she was very sympathetic to the back and forth Ventricle Measurements and assured me she would personally remeasure everything and update the reports accordingly. She did exactly that which already puts her far ahead of anyone I interacted with at OHSU. The schedulers also bother to call within a reasonable amount of time to setup appointments which is a nice change.

 

[Chuck C]

I wonder what that sugeon at Cedars-Sinai was seeing/thinking. I guess it makes sense that surgeons just want to do surgery hah.

I'll give a little more context, and I think it only fair to the Cedar Sinai surgeon that I do so.

When I consulted with him I had completed an echo a few weeks earlier. Yes, it had me at moderate AS, but my valve area was 1.0cm2, putting me at moderate, but right on the border of severe. My MPG and peak jet velocity also agreed with moderate. But, I also was diagnosed with mild left ventricular hypertrophy (LVH). My local cardiologist felt that I was many years away from needing surgery, based in part with how I set a record on the treadmill test, so it was a stunner to hear the surgeon tell me that he'd like to schedule me for next week.

I had read up on the LVH by this point, and was aware that many athletes develop mild LVH, known as Athlete's Heart. I had always pushed myself very hard in cardio fitness all my life, and I wondered if this could be partly to blame for my LVH. The athlete version of LVH reverses once the intense athletics stop, over the course of months. As far as I am aware, it is not a medical concern, as it does not progress beyond a certain point and reverses once the demands of sport are ceased. I speculated that my LVH might be from a combination of my hard cardio training and my AS. I discussed with the surgeon that I could ease up on the sports and see if this impacted my LVH. He indicated that my LVH was almost certainly due to my AS and that this would not make any difference, in his view. He also said that we could wait another 6 months and get another echo if I wanted, as it was not critical to for me to get surgery right away, it would be just his recommendation not to wait, so that things did not progress.

A couple weeks later I got another cardiology consult at Scripps. He was somewhere in between the other two opinions. He indicated that my LVH was not close to the point where it was likely to be irreversible and also estimated that I would need surgery within about 2 years. Future consults at UCLA would agree with his assessment.

As for the LVH, I backed off sports completely for about 6 months, and only did brisk walking. I also lost weight, bringing my blood pressure from about 130/80 to 115/80. It turns out that my plan was successful and I did, in fact, reverse my LVH. My second echo found some LV enlargement, but just below the LVH threshold. Then 3 echos followed in which no LV enlargement was detected and no LVH.

So, I can say that I'm glad that I sought several opinions. My local cardiologist, while very conveniently located near me, would have likely held on to me way too long before referring to surgery. He once shared with me that he had patients with mean pressure gradients at over 90mmHg who he had not yet referred to surgery. Yikes! I know now that this is a real bad idea. His view was to wait as long as you possibly can in the hopes of the development of new technology. I'm of the view now that he was way off the mark.

I guess it makes sense that surgeons just want to do surgery hah.

It's funny that you should say this. There is a grain of truth to this, at least with some surgeons. I met with two cardiologists at Scripps. When I brought up how the Cedar Sinai surgeon wanted to operate and not delay, they both joked about how "Surgeons want to cut." Apparently, this is a saying among cardiologists. On the other side of this coin, many surgeons feel that some cardiologists wait too long to refer their patients for surgery. I recall a video presentation by the well known Lars Svensson and Doug Johnson at Cleveland Clinic. Lars had just operated on a patient whose cardiologist waited until his ejection fraction had dropped to 25% before referring him to surgery. He said that full recovery was unlikely and spoke some on how some cardiologists wait too long to refer to surgery, resulting in poor surgical outcomes.

I had a lot of consults in the 20 months leading up to my surgery. I think that there is some truth in the cardiologists saying about some surgeons being eager to cut and merit to the criticism of the surgeons who feel that some cardiologists wait too long to refer. I felt my surgeon at UCLA was not overly eager to operate. He consulted with me when I was moderate AS also, but felt it was not yet time. Also, although my local cardiologist most likely is in the camp of holding onto his patients too long, I did not feel that this was the case with the cardiologists I consulted with at Scripps or UCLA.

I guess this is a long way of saying that it is generally a good idea to get second opinion and even a third with respect to timing lol.

 

[pellicle]

don't know anyone in my life who has had a valve replacement or even OHS in general.

This is pretty common. Even now I can only say that I know one other (who I've known since childhood), he was diagnosed a few years back; so exactly this place is good for meeting "family".

I don't know how much lurking you did here before joining but I'm also on a mech after my 3rd OHS. Mine were sprinkled throughout my life with first at 10yo, 2nd at 28, 3rd at 48 (which was driven by an aneurysm). I didn't read everything word for word that Chuck said, but I've little doubt I'd agree entirely.

What I didn't see mentioned was the issues one will face in dealing with the establishment. Being an Australian (Colonial) I get to do what I want pretty easily, and in Australia what amounts to no more than a Blood Glucose Meter (that measures different things about the blood) is available to buy (and indeed is distributed for free by certain valve makers, although this is not publicised to anyone except the surgeon). I know of one other Australian (also from Queensland) who got such a thing.

I read here how much difficulty many go through to manage themselves in the USA, there are some exceptions (I'll resist naming them) and Chuck is one; therefore it can be done.

Ultimately if you do go down that path (mech and self managing INR) then please do reach out and I'll help you on a good path of self reliance in as fast a manner as is reasonably expectable.

Lastly I can only but agree about the Ross and quite a number of people here have reported that after 20 years they regretted it. At 20 years out you'll be 48 and we'd hope still being full and active in your life.

I recommend also (if you haven't) settling in and going through these two presentations:


and then followed up by this one to answer further questions



Also this discussion is worth listening to as well
https://www.medscape.com/viewarticle/838221
Best Wishes

 

[Deidra]

Ultimately if you do go down that path (mech and self managing INR) then please do reach out and I'll help you on a good path of self reliance in as fast a manner as is reasonably expectable.

Thanks so much for sharing all the knowledge and resources you have! I did lurk quite a bit and read some things on your blog which were very informative in terms of valve selection and INR management. I just finished watching these videos too. It's really amazing to me how much mechanical valves have seemingly fallen out of favor despite the many advantages associated with them. I can't imagine going for a tissue valve in my position knowing everything I do. The testing and ticking seem like very minor downsides compared to guaranteed more open heart surgery with the Ross or tissue valve. Plus I already take a bunch of pills everyday so one more isn't going to make a big difference heh.

I'll definitely reach out if/when I end up with the mechanical valve. Best wishes to you too

 

[Deidra]

I guess this is a long way of saying that it is generally a good idea to get second opinion and even a third with respect to timing lol.

I agree with this, and I'm glad your situation worked out well at UCLA. Waiting until 25% EF before surgery is wild. My cardiologists are concerned with 54%, and I think they're right to do so.

It's interesting you mention the sports/physical activity. I similarly was very active in strength/endurance sports and training around the time the dilation and regurgitation were first caught. I started managing my stress better, held off on doing anything other than walking and jogging/biking for the next few years + got put on Losartan and now my LV mass and dimensions have gone down a noticeable amount since then.

 

[pellicle]

Hi

Thanks for the kind words.

It's really amazing to me how much mechanical valves have seemingly fallen out of favor despite the many advantages associated with them

Yet, if one discusses exactly this one is criticised as tissue bashing or having an Anti Ross Agenda.

In my own history I have had a repair, a homograft and a mechanical, so my experience with alternatives to mechanical is that on a long enough time scale the durability of the others drops to near zero.

Depending on what your durability criteria are and other medical reasons for contraindication, other options may be a better choice.

Best Wishes

 

[pellicle]

@Deidra
This is just one more presentation by Dr Schaff

 

[dick0236]

Edit: This is mostly unrelated but a fun fact I just learned. Apparently Dr. Albert Starr was director of cardiac surgery at OHSU until the 1990s, and the first Edwards-Starr valve was implanted into a young woman at OHSU.

Hi Deidra, welcome to the forum. Dr. Starr, along with Lowell Edwards (electrical engineer) designed and built the first commercially successful mechanical heart valve in 1960. It was implanted into a young woman (33) who died 10 hours post-surgery from surgical complications. The second recipient of the valve, Philip Admunson, lived 15 years and died after a fall from a ladder while painting his home. I have one of those first generation Starr-Edwards aortic valves that was implanted in me on August 16, 1967, that is still ticking after 56+ years....and, at age 88, I can't say the same about the rest of me.

 

[Lucker]

I already take a bunch of pills everyday

Could you please tell what are those pills? Your profile info says only losartan.