Showing posts with label Aortic regurgitation. Show all posts
Showing posts with label Aortic regurgitation. Show all posts

Monday, October 12, 2015

Decisions, decisions ...



Almost three years ago, I accidentally discovered that I have severe aortic heart valve regurgitation (leaking). The only treatment for this problem is surgery, the timing of which is somewhat controversial because we lack the highest quality evidence - multiple randomized controlled rials - addressing that question. Nevertheless, there is reasonable evidence suggesting that the time to operate was not then, and I have been grateful for the three pretty normal years that followed. Unfortunately, it appears that that time has come now.

My left ventricle has shown subtle signs of ongoing dilatation on serial cardiac MRI examinations and that volumetric increase was quite clear on my most recent images. Delaying operation has some benefits that seem largely static with the passage of time while the risk of adverse outcomes increases. The recent dilatation in my ventricle suggests that I'm probably at or near the inflection point of the curve relating the risks and benefits of waiting. It's impossible to know for sure, but I've always wanted to err on the side of operating little bit sooner rather than later if an error had to occur. Accordingly, I am going to wait no longer; my surgery is being planned for early in the new year. But no sooner is that question answered to a satisfactory degree that another question arises: what operation should I have?

The Options

Mechanical valve replacement
This option refers to surgically replacing my aortic valve with one made out of pyrolytic carbon discs that tilt open and closed. The advantage of mechanical valves is that they are very durable. There is probably an 80-90% chance that a mechanical valve will last me the rest of my life. The disadvantage of mechanical valves is that they are foreign material, and the body tends to form blood clots on foreign material exposed to the blood stream. If a blood clot formed on the tilting discs, it could prevent the discs from opening or closing properly. The valve could become severely blocked, impairing the flow of blood out of my heart, and causing a life threatening situation. Alternatively, the clot could break free and travel through just the right blood vessels leading to my brain, causing a stroke. To minimize these clot-related risks, an anticoagulant medication called warfarin is indefinitely required. Because warfarin interacts with changes in diet, liver function, and many medications, regular blood testing to determine the adequacy of anticoagulation are indefinitely required. Thankfully, I could perform these tests at home, about once a week, for the rest of my life. Despite good control of my use of warfarin, I'd probably be facing a 25% life time risk of life-threatening bleeding in the worst case scenario, or enough bleeding to require a blood transfusion in the best. So while there's probably a 75% chance of not having a major bleed, mundane human experiences like a bonk on the head, a car accident, or a bleeding ulcer could all pose serious risks to my well-being.

There are a couple of other considerations regarding mechanical valves. Firstly, they make an audible clicking sound when they close. Sometimes it's loud enough to hear in a quiet room when standing next to someone with a mechanical valve. You can check out what the click sounds like here. Some people are bothered by a sound that they can never get away from, while others get used to it and still others find it soothing or comforting. I'm not sure how I would handle the perpetual click, but I do know that if I can hear my watch ticking on my night table, I can't sleep until I put it away. Secondly, ultrasound examinations of the blood vessels of people with mechanical valves often reveal little specks flying around in the blood stream. These are thought to represent very small blood clots or bubbles, and since they are flying around all over the place, some go to the brain. There is some evidence that patients with relatively high numbers of these tiny emboli have deficits on detailed neurocognitive testing (such as memory defecits). It is unpleasant to speculate that a mechanical valve could lead to early onset dementia. Some biological valves (see below) are associated with these micro emboli, too, though they seem more common among people with mechanical valves.

Biological valve replacement
This option refers to surgically replacing my aortic valve with a new one made out of biologic material from a cow or pig heart. The tissue is treated so as to not invoke an immune rejection response. The advantage of biological valves is that they do not require anticoagulants after the first 3-6 months post-op. The major bleeding risk is therefore about half of that observed with mechanical valves. The downside of biological valves is that they wear down over time and that rate of structural deterioration is fastest in younger patients. A biological valve would be expected to last 10-15 years before requiring replacement surgery, so there is a very high chance that I'd have to have a second open heart operation to replace a biological valve in my lifetime, perhaps, even a third. While the risk of bleeding is lower over those years, the risk of the second surgery is probably double the risk of the initial surgery (though we're still talking about relatively low risks), and if a third operation is required, the risk seems to really increase significantly. Plus, each operation also carries a risk of neurocognitive decline just from being on cardiopulmonary bypass for a few hours. Open heart surgery is basically the mother of all operations, taking 8-12 weeks from which to recover. Nobody wants to have to undergo it more than once if they don't have to...

Mechanical and biological valves compared:
Both mechanical and biological prosthetic heart valves carry a similar and, thankfully, very small risk of becoming infected (endocarditis). This is a serious complication, often requiring redo surgery to remove the infected valve and replace it with a new one. At the end of the day, when these valves have been compared in people over 55, the risk over time of stroke is the same, though mechanical valves require anticoagulants for that to be the case. Survival has seemed pretty much the same, too. With a mechanical valve, the risk of bleeding is spread out over time, while with a biological valve, the risk is concentrated at those times when the valve degenerates and re-operation is required. However, there have been no randomized trials comparing mechanical and biological valves in people in their 40's, so the bottom line is that nobody can provide any confident information about how someone like me ought to expect to fare with either. All one can do is make an educated guess based on trials involving older patients and the best guess of some experts is a mechanical valve, while for others, its a biological one. (Hmmm, an interesting and important disagreement.)

Valve Repair: There is a small chance (estimated at 20-30%), that my aortic valve could be surgically repaired. This would mean that the surgeon could, if the tissues are strong and the valve leaflets long enough, artfully put in some carefully places sutures that would re-align the leaflets and make them close well again. Like a biological valve, repair would have the advantage of not requiring anticoagulants, but more importantly, it would not be associated with an increased risk of infection because no foreign material is involved. The coolest thing about a repair is that while prosthetic valves just palliate the problem, a repair could potentially cure it. The downside of a repair is that its longevity would be unknown. It could last my lifetime, but it could also fail within 2 years and require redo surgery that soon. One estimate is a 20% chance of needing a second operation at or before 10 years. The trouble with these estimates is that the cause of my valve leak isn't known; the usual conditions that cause it have been excluded. Maybe I have weak connective tissues and a repair would be destined to fail again just as my original valve did?

What am I going to do?
Well, I've made a choice, but it's not like I feel very confident about it. I remain open to new evidence and other opinions. At this moment in time, I'm planning to have a surgeon who is very experienced with aortic valve repair - and there are not that many in the world - make a judgement call in the OR: if he thinks that there is a good chance for a durable repair, that is my #1 choice. If he feels otherwise, my second choice is a mechanical valve. The On-X aortic valve has recently been shown to be as safe with less aggressive anticoagulation (INR 1.5-2 rather than 2-3) and therefore less bleeding, so I have asked my surgeon to use this valve if it comes to that.

Repair is attractive because of the lower risk of endocarditis, and because it represents a chance to be normal again. I'm willing to risk something for that, but not much. If the surgeon thinks my tissues are not up to the job of providing me with a lasting competent aortic valve, then I would prefer erring on the side of implanting a mechanical prosthesis. Why mechanical? Because while survival of patients with mechanical and biological valves has been similar in trials of older patients, my suspicion is that there will be a survival advantage in younger patients because the risk of bleeding - the major risk of a mechanical valve over biological - is lower in young patients. Plus, I'm choosing a valve that likely requires less intensive anticoagulation than has traditionally been required, and I would monitor my anticoagulation at home. All of these things should help to reduce the risk of bleeding with a mechanical valve during young years when a biological valve would be undergoing relatively rapid structural deterioration. Also, I somehow feel more comfortable taking my chances with anticoagulants, which I can to some extent control, than with watching and waiting for the clock to tick down to a second big operation. (That doesn't seem like a particularly rational consideration, but I'm with David Hume, whom I must quote at every opportunity: "Reason is, and ought only to be the slave of the passions, and can never pretend to any other office than to serve and obey them.")

I wish that there was an ongoing randomized controlled trial comparing mechanical and biological valves in 40 year-olds with long term follow up. I would gladly participate in such a trial and let the study make that decision for me. Unfortunately, and for reasons that are really unclear to me, no such study is underway, nor, to my knowledge, even being planned. It would seem to be so easy to do, and it's a question that's important to many thousands of people like me around the world. Too bad.

So that's likely going to be my roll of the dice. I'll probably end up with a mechanical valve and the best case scenario is that it never gets infected (95% chance), that I have no major bleeding while on warfarin (75% chance), that I get used to the click and even come to like it (? chance), and that it lasts me a long life (80-90% chance) without major neurocognitive decline (? chance). Of course, this discovery has forced me to rethink the concept of a long life: my average life expectancy is probably somewhere around 65* while it ought to be well over 70. And while it would be nice to make it to 75, doing so with cognitive dysfunction represents a particularly unattractive scenario to me. Hopefully, safer alternatives to warfarin will become available for use with mechanical valves in my lifetime.

But like I said, I'm open to other perspectives. Please share your thoughts in the comments below. Would you take a chance at valve repair? Would your back-up plan be a biological valve with a high chance of a second operation, or a mechanical valve with the life-long use of warfarin? How would you roll the dice if you were in my shoes?


*Martijn W.A. van Geldorp , W.R. Eric Jamieson , A. Pieter Kappetein , Jian Ye , Guy J. Fradet , Marinus J.C. Eijke... Patient outcome after aortic valve replacement with a mechanical or biological prosthesis: Weighing lifetime anticoagulant- related event risk against reoperation risk. The Journal of Thoracic and Cardiovascular Surgery Volume 137, Issue 4 2009 881 - 886.e5

Wednesday, March 5, 2014

New Guidelines for Managing Valvular Heart Disease Are Out



The new ACC/AHA Guidelines for Valvular Heart Disease are just out:

It seems that I am on the cusp of periodic surveillance and a Class IIb indication for elective valve replacement.

Sunday, March 2, 2014

News about my health


About a year ago, my second child was two, and while what I had was adequate, I decided to get some additional term life insurance since it’s still relatively cheap for the young and healthy. You never know.

Then a nurse who visited my home as part of the underwriting process recorded my BP at 150/60.

What? My BP had always been around 115/60-70.

She wasn’t that hot.

“Relax,” she said, “lie down and relax and we’ll take it again.” The trouble was, since I had had nothing to worry about, I had been relaxed, though after that reading, not so much.

We eventually were able to get a couple of readings at 130/50, and the rest of the interview and exam went well even though I was a little distracted by the high systolic BP and relatively low diastolic BP. As a cardiologist, I got to thinking about a heart valve problem that can cause that precise BP pattern: aortic valve regurgitation (leaking). I got out a stethoscope, and along with the first beat I heard when I put it on my chest, there was a harsh, loud systolic murmur accompanied by a loud diastolic murmur.

“Where did that come from?”

I kept listening, beat after beat, wondering if what I was hearing was for real, but it didn’t go away.

Fuuuuck. (Sorry for the technical doctor-talk)

The office right next to mine at Foothills Hospital is occupied by a supremely talented and bright cardiologist with expertise in heart imaging. She arranged an ultrasound and MRI: aortic regurgitation (AR) is graded as mild, moderate, or severe, and mine was severe. Boom.

The other thing we learned was that this had been going on for at least several years since the extra blood volume rushing back into my heart had caused it to enlarge.

Severely.

Buh-oom.

The good (?) news was that my heart pumping function was still normal, everything else looked fine, and I had no symptoms. In fact, I had trained and run my first 10k race about 6 months earlier and had been running 5k every morning in Maui just a couple of weeks earlier. I wasn’t in the best shape of my life, but I felt pretty darn good.

Questions arose and played a rather constant game of musical chairs for a seat in my consciousness. How had I not noticed this before? Why was my valve leaking? Why me? Can my kids get this? Will I live to see 50? 60? 70? Tomorrow? And of most concern, because AR is a mechanical problem that requires a mechanical solution, namely valve surgery, when would I need to have mine?

There was an easy answer to the first question. Aortic regurgitation can progress slowly over time and the heart compensates by enlarging to accommodate the blood that leaks back with each beat. The net forward blood flow remains normal, so symptoms are rare until it’s been around for a long time. As a cardiology resident in my twenties learning how to do and interpret heart ultrasounds, I had done several on myself and my aortic valve was normal then, so this had to have developed sometime in the past 20 years. Diastolic murmurs are rare and challenging to hear, so it’s no surprise that my GP hadn’t picked up on it despite annual or biannual check-ups that I’d been having since I was 40.

There is a list of well-recognized causes of AR. The most common culprits for a young person include a previous infection that affects the valve, being born with a bicuspid aortic valve (which is a valve with 2 leaflets instead of the usual 3), or dilatation of the aorta so that the leaflets stretch apart and no longer touch in the center. None applied to me. To this day, the cause remains unknown.

Timing of surgery in severe AR is a question that doesn’t have a clear answer either. Thankfully, there are reasonable ones. The concern is this: as the heart enlarges to compensate, there reaches a point where irreversible heart dysfunction begins to develop. The enlarged heart can become weak, not squeeze well, and if left long enough, that may be permanent. One might ask, why not operate right away in that case, so as to not permit the mistake of waiting too long to occur. Unfortunately, the surgical procedure and options are far from perfect. The operation itself has a risk of death that approximates 1%, and the best solution is to replace the leaking valve with either a pig valve or a mechanical one. Pig valves don’t often last > 10 years in young people, so once the first surgery is done, a clock starts counting down to the timing of the next valve replacement operation. A mechanical valve is likely to last for many decades, but the foreign material is prone to blood clots that can cause life-threatening valve blockage, or, if the clots break free and get pumped into and block a brain artery, stroke. Oral anticoagulants (OAC) are forever required to prevent these clot-related complications, and they increase the risk of bleeding, which, while small, builds up over the years. Have a car accident on OACs and serious bleeding can turn fatal. Bang your head on OACs and a goose egg can turn into a coma like the one Michael Schumacher has been in for over 2 months. So there are a number of good reasons to try to put off valve surgery as long as possible.

Back in the 80's & 90’s, there were even better reasons to put off valve surgery: the risks of the operation were considerably higher then. Not knowing when the optimal time to intervene and best preserve longevity and heart function, cardiologists made the decision to operate when the patient developed symptoms like shortness of breath, weakness, fatigue, and exercise intolerance. Thanks to several groups who performed careful long term follow up of consecutive patients with AR and reported their results in medical journals, some patterns began to emerge. As long as valve replacement occurred before the heart dilated beyond 55 mm during a contraction, or beyond 75 mm during relaxation, and before the pumping function had dropped below 50% (normal = >55%), nearly all patients had normal heart function after surgery. The latest guidelines recommending these cutoff values for valve replacement have been around since 2006, and updated ones are due any day now. My heart pumping function was 53-55% and my heart measurements were 43 mm and 63-65 mm respectively, so I hadn’t reached the cut-off values yet.

Still, the opinions of my cardiologist and others who had been involved in imaging my heart were mixed. While they agreed that surgery wasn’t urgently required, some advised making plans to operate within 6-12 months while others suggested waiting and watching the measurements with repeat ultrasounds every 6 months. My initial inclination was also to get a new valve sooner rather than later. I just didn’t like the idea of having the leak and knowing that it was causing loading conditions that were slowly changing the number, size and function of my heart cells. I was, after all, getting close to the cut off values anyway.

An ultrasound done 3 months later showed no changes, so we knew we weren’t dealing with a rapidly progressive process.  Long story short, I just had my one-year follow up ultrasound and it looks exactly the same. While the first 6 months after diagnosis were pretty stressful, all those circulating questions started slowly receding and I found myself gradually able to return to thinking about the usual kinds of things that I think about, including making plans for the future. Those were all put on hold for a while. My appetite returned to normal, but my diet improved. I kept exercising aerobically and feeling well. I made a lot less Lego.

Life is pretty much back to normal now, except for that common cliché that certainly applies to me: everyday, I wake up and feel lucky. There is an equalizing comfort in the knowledge that all that anybody has is this precious present.

I’ve seen a world class valve disease expert who is the lead author of the updated joint American College of Cardiology and American Heart Association valve disease guidelines. He’s followed many patients like me for decades, and he tells me that I may be able to avoid surgery for years. In the meantime, I’ll get ultrasounds every 6 months to carefully watch for any changes, and I’ll hope that more durable tissue valves, or more clot-resistant mechanical valves, or better OACs get developed. It’s pretty much business as usual again in our house.

I find myself wanting to share this experience with others, and wanting to keep a record of some of the things that are important to me as I move on. If the valve should somehow catch up with me sooner than expected, I like the idea of having a blog that my kids might be able to read and at least kind of know me by. That’s one of the hardest things to imagine, that I might die while my kids are too young to remember me, or to be influenced by me in a more-than-genetic way.

Remember that insurance that I applied for? Ironically, I was approved at a preferred rate for especially healthy and low risk clients. How d’ya like them apples?


Of course, I had to decline the insurance because of what I had discovered during the underwriting process, which is fine because I’m adequately insured anyway. If you have kids and you’re not, I strongly advise you to at least get some cheap term insurance while you can. Our bodies evolved on the African savannah over millions of years. There is no evidence of any intelligence in the design- only the pressures of reality selecting among random trial and error. That’s why we break down all the time. It’s amazing that I haven’t had any health issues until this and I hope that you’re all considerably luckier than that, but one shouldn’t count on it or take that risk with the well-being of one’s dependants. There is no magic keeping anybody safe. The sun rises and the rain falls equally upon us, so let's make sure that we have sunscreen and an umbrella.