1997 counting crows at pinkpop - long december

"The smell of hospitals in winter..."

Avoiding Permanent Heart Damage

I've been thinking about what I read recently...a detailed description of what happens to your heart when your aortic valve becomes severely stenotic (stenosis means "narrowing"). At the moment I can't locate the more technical description, but this from the Mayo Clinic web site gives you the general outline:

If the aortic valve is narrowed, the left ventricle has to work harder to pump a sufficient amount of blood into the aorta and onward to the rest of your body. In response, the left ventricle may thicken and enlarge. At first these adaptations help the left ventricle pump blood with more force. But eventually these changes weaken the left ventricle — and your heart overall.

I'm going to look today for the technical description of what happens. It has something to do with blood supply to the heart which becomes disrupted or irregular as the heart enlarges in an attempt to compensate for the narrowed valve. This results in ischemia of the heart...which is defined as a localized anemia of living tissue. In other words, parts of your heart begin to suffer from lack of blood supply...therefore, lack of oxygen, as I understand it.

So this is why you don't want to wait to long to have valve surgery if you need it. This sort of damage is NOT reversible, according to what I've read.

The Nature Of Bicuspid Valve Disease

As I wrote to my new friend Jane, who also has a bicuspid aortic valve, I have found out that BAV disease is actually a syndrome, with associated connective tissue disorders, that affects other things in your body, like your arteries, and is even associated with other physical manifestations like nearsightedness, which I also have. In this respect it is in some ways similar to other systemic conditions such as Marfan"s Syndrome. It's not merely a problem confined to the aortic valve. BAV disease appears to be something that is not well understood by doctors, even cardiologists. It's a cutting edge sort of condition. With BAV, you should be screened for the condition of your arteries: your thoracic aorta, your carotids, you should get a brain scan, and a scan of your abdominal aorta. Aneurysms can occur in any of these places.

I'm not anxious to have the surgery on the one hand. On the other hand I want it if it's time, and before there is permanent damage to the heart, and before I blow a gasket and have an aortic dissection or rupture. I want this for myself, and for those I love and who care about me.

Confusion, And The Holiday Food

There's so much information to digest and remember. You read all you can...or at least I read all I can...but then it's like information overload. Stuff starts falling off the shelves in my mind. I have to go back and re-read and try to organize my thoughts once again. Where do I stand? What do the numbers mean?

I have a friend who I met through our shared interest in toy train collecting. He's an anesthesiologist. He found out about my heart condition and kindly wrote to me. Turns out he was in training to be a cardiothoracic surgeon before changing career paths. He wanted to know about my condition and offered advice.

So I told him about the BAV diagnosis and the aortic aneurysm or dilation (some people like my GP don't seem to be up on the current literature that lists anything over 4 cm as "aneurysmal.) I also explained that I would not agree to surgery unless the battle plan also addressed the aneurysm. He wrote back saying that my surgeon might not want to repair the aorta because the dilation was probably due to stenosis of the AV.

Of course, my friend has probably not read the current literature on BAV disease and the studies that indicate that aortic dilation is separate from BAV stenosis, happens due to causes other than BAV stenosis, and will progress in a significant number of patients even after BVAVR.

Through all of this I'm trying to keep my eye on the ball because I was to have a clear head when I meet my surgeon on January 6. This is what I have to keep front and center:

In patients with bicuspid valves undergoing AVR because of severe AS or AR (see Sections 3.1.7 and 3.2.3.8), repair of the aortic root or replacement of the ascending aorta is indicated if the diameter of the aortic root or ascending aorta is greater than 4.5 cm.* (Level of Evidence: C)

This is from the 2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease, the very lastest practice guidelines. My ascending aorta is a 4.3 cm as of the lats measurement in November. If the surgeon "pulls the trigger" when I see him on January 6, the plan has to include tackling the aortic aneurysm because I'm only 2/10ths of a CM under the trip point in the guidelines. If not, then would not agree to surgery because of the risk that I would have to undergo a second OHS in a few years. So we'll see next Tuesday after I get the CT scan and meet with Joe Bavaria.

In the meantime, I'm walking four miles a day usually, and trying desperately not to consume too much of this tasty food that is all to readily available around the holidays. I made a meal for seven on Christmas day and the leftovers are delicious. Yesterday Dan made a turkey pie and we had some of it for dinner. It is really wonderful. I made holiday cookies last week, Viennese Crescents, and they're yummy too. How do you not put on a few pounds at this time of the year with all of this good food around?

Another Interesting Story

This was posted on VR.com by Andy, a fit young man who lives in Australia. It is typical of the kinds of stories you read about people with heart valve disease:

My story seems quite common here: an asymptomatic, fit and otherwise healthy person suddenly finding out they have heart disease which needs fixing soon. Life is turned upside down; mortality suddenly becomes very tangible. I’m 42 and have exercised virtually daily for many years (gym, swimming, cycling, walking etc). In August 2008 I fainted while swimming (at the end of a final lap, sprinting as fast as I could). The fainting episode must have been brief as I didn’t take in much water (or drown for that matter!). While passed out as I was having a nice little dream (I can’t recall the details now but it was pleasant). It was thus something of a shock to come to underwater, out of breath and unable to breathe, rather than waking up warm in my bed. Even though I’d not fainted before I put off seeing a doctor for a month or so; he soon identified a murmur with the stethoscope and sent me off for an echocardiogram. I didn’t get the result until some weeks later, but the news relayed by the GP was grim: ‘I’m sorry to tell you this on your birthday, but I’ve spoken to the cardiologist and we think that you’ll need mitral valve surgery; don’t do any strenuous exercise’ (he knew that I went to the gym regularly).

That feeling of "this can't be happening to me" is quite common, especially in asymptomatic people who exercise regularly and stay healthy. That's why valve disease is sort of a cruel diagnosis. I'm lucky to have made it to 60 with a BAV. Many people don't make it that long before they need surgery. Still, while I consider myself lucky in one respect, I'm not happy about it. However, eventually everybody has some medical problem to deal with...so at least I may be able to get a fix that will buy me some time.

Kittys And Sleeping After OHS

One of the challenges after you return home following OHS is how to get good sleep. The problem is that the incision makes it difficult to get in and out of bed, and sleeping positions can be painful. One idea that is used by a lot of people is sleeping in a recliner. Apparently, this makes sleeping and, in particular, getting up a lot easier. Of course, you have to have a recliner, which I don't have.

Here's a cute story about cats and sleeping post-op in a recliner or in bed from one of the VR.com members:

While I used the recliner a lot during the day, I couldn't sleep in it due to being visited by cats (some rather more than 10 lbs.), I slept mostly in bed. Back sleeping resulted in snoring loud enough to wake me up, so Laura used various wedges of blankets, towels, and pillows to position me for comfort--about halfway between back and side. On cold nights she would also microwave a buckwheat pillow for me, and I would use it as necessary for warmth (heat lasted for hours).Strangely, it was a heart-related incident that forced me to use a recliner. Back up in Anchorage in 1996, shortly after I noticed a rather abundant number of PVCs during our very stressful move and drive to Anchorage from Idaho, I wore a Holter monitor for 24 hours to record my heart action awake and asleep. I couldn't lay down in bed, since the device was too cumbersome, so I wore a bathrobe, placed the main monitor into one of its pockets, covered up in my Lazy Boy recliner, and managed to get a reasonably good night's sleep. There were cats then, too, but I could take a nighttime 'visitor' or two--just shoved him or her off when too warm or heavy. Amazingly enough, three of the six cats we had then are still alive, including one that's now 18--and she's in great health. Of course after the OHS, circumstances wouldn't allow me to free myself of heavy cats, so I couldn't take the risk. Anyway, Laura made the bed nice and comfortable, and it made her feel a lot better to be beside me in bed--and me, too.

Chris

High Blood Pressure & Valve Disease

So, I'm trying to figure out why my blood pressure is creeping up. It appears that there is a connection between aortic stenosis and hypertension:

Systemic hypertension is not rare in patients with symptomatic aortic stenosis (32% in our series). Left ventricular remodeling patterns are quite similar in hypertensive and normotensive aortic stenosis. Our results suggest that symptoms of aortic stenosis develop with larger valve area and lower stroke work loss in hypertensive patients, probably because of the additional overload due to hypertension itself. It could suggest that in patients with coexisting hypertension and aortic stenosis, hypertension should be treated more aggressively to delay the occurrence of symptoms, and these patients should be followed-up more closely.

Of Hills And Christmas Dinner

I did a quick walk yesterday afternoon before our dinner guests arrived. On the way back I took the route that requires an uphill walk up a ramp. I sure felt that, and not in a good way, with a little chest tightness and SOB.

For Christmas dinner I cooked a vegetarian entree: Tofuturkey. I've tried the stuffed tofu substitute poultry entrees that are available at our local Whole Foods and I like them. So we had something that didn't have to die available for those so inclined, in addition to the traditional "dead bird" that we're all used to. Three out of seven opted to go vegetarian, which is also heart healthy.

Asymptomatic But Needing Surgery

This was posted recently on VR.com:

I am an active 44yo male with AI diagnosed in childhood. I have enjoyed annual checkups and echos (until this year). Recently I visited my cardio for my exam and he now informs me I have progressed to "severe" and surgery is needed. I am going for a second opinion in early January. I have always known that surgery is immanent but now? I feel great exercise pretty regularly, and am very active with no limitations. I have always been told what symptoms to look for and I have none
of them.

It's amazing how you can walk around and do most anything even though your valve is in bad shape. If you are in good physical condition, the body adapts and this can fool you. I'm in the severe range myself with a bicuspid aortic valve and a 4.3cm ascending aortic aneurysm, yet I feel fine and can do most anything...well, I get winded and I can't push to hard or else I get chest tightness. But I don't feel like I need surgery even though I do.

Blood Pressure Wars

When I was younger, before my AV went bad, I used to have really good blood pressure, e.g., 100/60. Apparently, as BAV disease progresses, you develop what is know as "labile" hypertension. That's when your systolic (top mumber) blood pressure rises quickly and substantially when you exercise, become excited, etc. I don't understand the mechanism, but labile hypertension is one of the symptoms of BAV. Yesterday I went to see my doctor to get some blood tests done. Of course, many people have increases in BP in the doctor's office. Mine was way high (for me,) something like 160/90. My usual resting BP is in the 130/80 range. Well, my days of not taking any pills are just about over. I'll have to go on some sort of BP medication ASAP. I really should be on it already.

BAV Disease & Surgery Timing

From the Cedars-Sinai web site:

Generally speaking, a slightly enlarged aorta in the presence of a bicuspid aortic valve (BAV) has a higher incidence of aneurysm formation and dissection. Unfortunately, there are no tests that can predict whether or not the aorta will continue to enlarge or be prone to dissection in the future. Because of this and the high mortality rate associated with acute ascending aortic dissection in those with bicuspid aortic valves, specialists in aortic surgery recommend that a slightly enlarged ascending aorta be replaced at the time of valve replacement.

On the CS website, the trip wire for surgery on an aortic aneurysm on someone with a bicuspid aortic valve is 4.5 cm. However, the above paragraph stipulates that for people with BAV, even a "slightly enlarged" aorta should be replaced at the time valve surgery is done. This is because people with bicuspid valve disease have a higher risk of aortic aneurysm rupture or dissection.

Now, my aorta was just measured at 4.3 cm, which is more than slightly enlarged, it's an aneurysm. Even if my aortic valve was just fine, which it isn't - it's moderately to severely stenotic - I would only be 2/10ths of a CM away from the surgery trip wire. So what's it gonna take for my surgeon to pull the trigger and schedule me for surgery? Not much, I don't think!

Two Weeks Until Decision Day

I've been following Scott's progress down in Texas. He had surgery last Thursday. Today he is finally getting out of the ICU and into a regular room. He had his second open heart surgery to replace a dilated aorta and root and they put in a new aortic valve. Probably because this was his second OHS, he needs a pacemaker to keep his heart beating properly. It seems like his recovery is on track, although he will require a longer hospital stay. His partner Byran will be staying in the room with him from this point on, according to what I've read on Scott's blog, so that should insure that all of Scott's needs are taken care of. I wish him the best and hope that he can be released maybe by the weekend.

I go to get blood work done today in advance of my CT scan scheduled for January 6. So, it's two weeks from today until I meet again with the surgeon and find out what the battle plan is. In the meantime, I'm still walking twice a day for a total of about four miles, even in the cold. Yesterday my AM walk was interesting because the wind was really blowing strong from the Northwest off the river, and the wind chill must have been about zero degrees. It felt like that anyway. I wear my heaviest parka, hood, gloves and "Ninja" mask to cover my face. The only thing that was cold was my legs. I could benefit from some "Long Johns" but they are hard to come by to fit me because of my long legs.

Anyway, I'm feeling good and I'll continue the walks, and continue living life as normal as I can until Jan. 6. I have only minor symptoms, the palpitations, and getting winded easily on hills and steps. No dizziness yet, or swelling of the feet, etc. But why wait for that to occur? My condition is not going to get better, only worse. Also, aneurysms don't usually have symptoms...until they burst or dissect...and then you're in BIG trouble.

So, January 6 will be the day of reckoning. We'll see what happens then and we'll take it from there.

Open Heart Surgery And Pacemakers

With OHS, after you get out of surgery you're hooked up with quite a few tubes and wires. One of the connections is a set of pacemaker wires that the surgeon puts in during surgery.

Here's an explanation of why patients undergoing open heart surgery require temporary pacemaker wires inserted during surgery:

After heart surgery or another life-threatening event involving the heart, it may be necessary to put in a temporary pacemaker to make your heart beat at a normal rate...One type of temporary pacemaker may be attached to the patient's heart during heart surgery. This type has wires which go from the heart through an incision in the lower chest to an external box that makes the heart beat normally.

As I understand it, while the wires are present after OHS, a pacemaker may not be needed if the heart beats regularly and the wires may be removed at some point during recovery, or a permanent pacemaker may be needed.

What Will The Surgeon Suggest?

So, I have a bicuspid aortic valve with moderate stenosis and a dialated aorta. I meet with my surgeon again on January 6 after I get my tests done. What will be his recommendation: wait?...or surgery? It's not clear cut, it seems to me. In the literature, there is a great deal of controversy about both the timing and the extent of heart valve and anuerysm surgery. Some studies recommend replacing the dilated aorta if it is over 5 cm, others are at 4.5 cm, and another is at 4 cm. Mine was recently measured at 4.3 cm. The valve is moderately stenotic. If I just had the valve problem in isolation, waiting until it reaches severe in terms of numbers could be the recommendation, except that I do have mild symptoms. The presence of symptoms usually triggers surgery. But I also have the aneurysm. Will the combination of the two seal the decision for surgery now? I'll just have to wait for January 6 to find out the answer.

Here's a paragraph from one paper in the Journal Of Cardiothoracic Surgery (Mar. 06) that addresses the BAV/aneurysm conundrum:

Another surgical approach suggested for the management of BAV, associated with a dilated ascending aorta less than 5.5cm is to carry out AVR with wrapping of the ascending aorta. This method has a low morbidity and mortality rate and was seen to decrease the risk of further dilatation, aneurysm formation and dissection [54,55]. More recently this procedure has been carried out using an external support made to fit the patient's aorta. This was done using digital information from magnetic resonance images to make a replica of the patient's aorta and then computer-aided design to produce the tailored graft [56].

Sounds interesting...a custom made aortic "wrap." I wonder if this will be an option for me?

The Unrecovery

One poor fellow on VR.com, who is a few weeks post surgery, wrote this the other day:

It gets depressing that every time I go in some doctor finds another problem. Sometimes I wonder if I will ever become normal again.

Seems like OHS is so traumatic...does so much damage to your body, that it takes months, even years to get over it, if you ever do. The "cure" is almost as bad as the disease, it seems. Well, that's just fine.

Knowledge Is Power!

Dan may sometimes think I'm too obsessive about researching my BAV disease and aneurysm. However, in the Dec. 18 New York Times there is a fantastic article written by a doctor that makes a powerful case for patients being as knowledgeable as possible about their condition. Here are some key quotes from the article:

Patients are more likely to spend time researching a job change (on average, about 10 hours) or a new car (8 hours) than the operation they are about to submit to or the surgeon who wields the knife.

“Today, medicine and surgery are really team sports,” Dr. Russell continued, “and the patient, as the ultimate decision maker, is the most important member of the team. Mistakes can happen, and patients have to be educated and must understand what is going on.”

“If we are truly going to reform the health care system in the U.S.,” Dr. Russell said, “everybody has to participate actively and must educate themselves. That means doctors, nurses, other health care professionals, lawyers, pharmaceutical companies, and insurance companies. But most of all, it means the patient.”

Another Concern: Germs And Colds

I'm just getting over a nasty sinus head cold. I'm also reading on VR.com about someone who is scheduled for surgery very soon, in a matter of days. Her surgeon told her to "live in a bubble." In other words, don't put yourself in any situations where you can pick up germs and risk catching a cold. You don't want to have heart surgery with a cold. Well, this is just one more concern to be aware of and to deal with. Get out the masks and the hand sanitizer!

Scott Made It!

Our friend Scott made it through complex surgery yesterday down in Texas. Scott has part of his aorta replaced as well ans a new aortic valve. We're pulling for him and very glad that he made it to "the other side of the mountain."

One New Valver's Comment Six Days Post-OP

I had other patients trying to cheer me by saying "just think you'll be able to walk to the shops or walk the dog again with this surgery". That may have been the case for them but I could have climbed Everest a week ago ! Now I'm out of breath having pulled my socks on ! What happened ...

What happened? You were hit by a truck, that's what!

The Transition: Life After OHS

I'm lucky, as someone pointed out to me recently, to have made it to age 60 with BAV disease. I've never liked taking pills or medications, and I don't take any even today. But that will all change soon, and that's part of what I really don't like about having to deal with my heart valve disease.

The other thing is the aftermath of surgery. Yes, having a diseased heart valve and an aortic aneurysm replaced is life-saving and should buy me a number of years, but it's far from certain that the quality of my life post surgery will be any where near what I'm used to. That's because of the kinds of complications people have after OHS. Yesterday I was reading about "TIAs" ( transient ischemic attack), which is like a mini-stroke, and full blown strokes that people get even if they are taking their coumadin and they have their INRs "in-range." Not good. Then you have to worry about getting endocarditis and careful with any invasive procedures like dental visits, colonoscopies, anything that breaks the skin. I read about so many valve surgery patients who must return to the emergency rooms at their hospitals with a variety of problems with their heart. Getting the surgery is no panacea.

Best Wishes, Scott!

Scott is having his surgery today. We wish him well and hope that his surgery is successful and his recovery smooth.

Labile Hypertension

According to the Bicuspid Aortic Foundation's web site:

The majority of those with BAVD have labile hypertension. Often their blood pressure goes up quickly under stress or when exercising.

I've noticed this phenomenon for some time now. My systolic pressure (the top number) goes way up if I go up steps or get stressed, like at the doctor's office. It makes it more difficult to get accurate measurements of what my resting blood pressure actually is. But that really makes no difference, because the goal is to reduce the blood pressure number both at rest and under exercise and stress so as to protect the aorta. That's why blood pressure management is so important in the treatment of bicuspid valve disease. I take my blood pressure two or three times a day. It's higher than recommended for people with BAV disease, so I'm sure I'll be placed on blood pressure medication before long.

Surgery This Week For A New Friend

I've made a new friend, Scott, who lives with his partner Bryan in Austin, Texas. Unfortunately, Scott has to undergo his second open heart surgery in just a few years. This is because he has BAV disease, and during his first surgery only his aortic valve was replaced. As is often the case with BAV disease, Scott has a dilated aorta and root that has now to be corrected. This should have been addressed by the surgeon at the time he had his valve replaced. We're all pulling for Scott as he undergoes surgery this Thursday. Scott is writing a blog which you can read here.

Scott's situation makes it very clear to me that I must have my aortic aneurysm addressed at the same time my bicuspid valve is replaced. I'm sure that this will be the battle plan that my surgeon will suggest on January 6.

Decompressing

After finding out last Monday that I have a bicuspid valve and an aneurysm, I had a rather intense week coming to grips with that reality and what it will mean - complex surgery to replace not only a defective aortic valve, but also part or all of my ascending aorta and root. I'm afraid I made Dan upset with an information overload that I dumped on him and I'm sorry for that. He thinks that I'm obsessing about it all, but I have to be on top of the situation to the best of my ability. I owe that to myself and him.

As of Friday, I began to come off of the jittery, panicky, drive to find out as much as I could by doing on line research. I'm pretty much over it now. Even my blood pressure is looking better these last few days. Unfortunately, I caught a bad head cold over the weekend. I started to feel it Saturday morning. Last night it was tough to sleep with the cold symptoms. I did get a walk in yesterday afternoon, which made me feel better. Our house is right across the street from Fairmount Park, so we enjoy daily walks with our dog Bradley. I actually do two walks most days: 2 miles in the morning and two miles in the afternoon. I only feel symptoms on exertion - walking up hills, steps, etc. If I do heavy exertion, I feel it in my chest, so my surgeon considers this "symptomatic." I'll continue my walking though, because I'm determined to stay in the best shape I can.

Being Proactive About Your Health

I had a great day yesterday. I kept busy doing fun things. In the morning I went to a model train show. In the afternoon I went out and bought a holiday tree and garland for the outside of our house. I set the tree up and put the lights on it and decorated the house. Last evening Dan and I spent a little time at a friend's holiday open house then we went out to dinner.. It was god to get back to a normal life after several intense days focusing on my heart problems.

I can't get over the fact that if it wasn't for my determination to take care of myself, I might be walking around today and not know about my condition. The whole thing started when I was 50. I didn't have to go in for that initial colon cancer screening, but I did. I had a polyp removed and that put me on the five year schedule for re-screening. When I was 55 I went in for another colon cancer screening. At that time the medical practice required that I have an echocardiogram first because of a heart murmur. When I had the echo, that's when I was told for the first time that I had a valve problem.

So I've been going for heart checkups since that time. My yearly echo this year showed significant progression of the valve disease. So I took it upon myself to get a second opinion - from a surgeon. That lead to the discovery of my bicuspid valve and aneurysm. One thing lead to another, and another, and another. But if I hadn't been proactive (I love that word!) about my own well being, I could still be in the dark about all of this, or worse, I could have colon cancer from an untreated polyp or dead from a burst aneurysm.

I've known people my age who refuse to go to the doctor to get checkups, etc. Many of them are ticking time bombs. It's no wonder people die at a relatively young age from conditions that could have been addressed, and their lives saved.

Nearsightedness..Ah Ha!

This is from the Bicuspid Aortic Valve Foundation:

Bicuspid aortic valve disease is more than a bicuspid aortic valve... Not everything is understood today, but enough is known to identify several major characteristics of this condition. BAVD includes the following:• Bicuspid aortic valve• Thoracic aortic dilation, aneurysm, or dissection• Labile hypertension (high blood pressure which changes quickly with stress or exercise)It may also include:• Mitral valve leakage• Coarctation of the aorta• Kidney or liver cysts• Near-sighted vision• Musculoskeletal degenerative changes (scoliosis, degenerative spinal discs, flexible joints, flat feet, etc.)• Aneurysm of smaller arteries, including head vessels (berry aneurysms)Not everyone has all of the listed characteristics. However, those diagnosed with a bicuspid aortic valve, who may have wondered why they also have high blood pressure, a leaking mitral valve, or poor eye sight, will recognize themselves in the definition above.

Well, that helps explain my nearsightedness. I've worn glasses becuase I am nearsighted since I was a child. I'm also struggling to keep my blood pressure at safe levels, and of course, I have the aneurysm. I'm grateful that I don't have any of the other characteristics and I hope I don't develop them.

The Bicuspid Aortic Foundation

One of the really great web sites for people with bicuspid valve disease is the website of the Bicuspid Aortic Foundation:

http://www.bicuspidfoundation.com/Index.html

There is a ton of great information here, including a page full of stories of survivors and tributes to those who did not make it. Last night I read the story of one young man who died in his 30s, leaving a wife who was pregnant at the time. He had a bicuspid valve and died of a ruptured aneurysm. It's a heartbreaking story but it's a testament to how insidious BAV disease is and how important it is to get screened if you have others in your family known to have bicuspid valves, or if you or other close family members have experienced unexplained chest pain.

The aorta is prone to dilation in people with bicuspud valves, and the the danger is that it can rupture, or "dissect," which means the layers can come apart and fill with blood. The Foundation is doing a great job trying to get the word out about this not well known and not completely understood disease. I went to another website yesterday, the Adult Congenital Heart Disease site, and I was surprised that they don't even list BAV as a separate congenital defect! I wrote them about this, and their president acknowledged that they probably need to address the problem.

The Lost Days

The past four days have been pretty intense. Since I found out that I have bicuspid valve disease and an aneurysm, I've been obsessed with researching the various elements of my situation. But I'm burned out a bit now and I need a break. Hopefully today I can put up our holiday tree, do some work on my toy trains...get back closer to a regular fun existence. It's been hard on Dan too, because I needed to bring him up to speed so that he has some understanding of things. I'm hoping we can have a pleasant weekend and de-intensify things a bit around here.

Aortic Regurgitation, Stenosis, Aneurysm

What does AV "regurgitation" mean? That's when the heart valve doesn't close all the way and some of the blood that the heart pumps each beat leaks back in to the lower chamber (ventricle) and the heart has to re-pump it. This puts additional strain on the heart. I have only mild regurgitation.

My aortic valve has a different primary problem: stenosis. This means that it is stiff and calcified, and the opening is reduced in size so the heart has to pump much harder to get the blood through the narrower opening. I do have severe level stenosis for my body size. This often happens with bicuspid valves. This will trip the surgery wire, I believe. My aneurysm, while significant, in and of itself would not be enough to warrant surgery at this time. What they would do if I had only the 4.3 mm aneurysm is to manage it with blood pressure meds and monitor it carefully with surgery only if it grew fast or larger.

But people with bicuspid valve disease usually end up having the valve replaced when it becomes severely stenotic. In my case, when surgery happens I will have the valve replaced as well as surgical repair of the anuerysm. This is complex surgery that really can only be done at a major thoracic surgery center like the U of P, and by an experienced surgical team, so I'm lucky. In order to do this surgery they not only have to use a heart lung bypass machine, but for a time, hopefully less than 40 minutes, they have to stop everything and there is no circulation of blood through the body. They can do this by inducing a state of hypothermia...cooling the body down drastically. When everything is stopped, they replace the part of the aorta that has the aneurysm with a sewn-in dacron graft. Then when they're done they put the patrient back on the machine or re-start the heart. It's amazing stuff and the survival rate is very good.

Good Information About Aneurysm Surgery

Here's an important study that applies to my situation exactly. It focuses on the prospects for patients with aortic aneurysms who have AVR surgery (that's me!):

CONCLUSION: Although the clinical course of patients with a dilated ascending aorta is unpredictable, aortic events may occur even in patients with a baseline aortic diameter of less than 50mm. Therefore, preventative aortic surgery at the time of AVR should be considered to prevent aortic dissection or rupture in patients with an even slightly dilated ascending aorta with a diameter of 40 to 50 mm, unless the patient has a high operative risk or older age.

Well, my aorta is at 43mm. So if the surgeon decides it's time to do an AVR, this would indicate that he would also do aortic surgery at the same time. This is actually a relief to me - to find this abstract. That's because I don't want to have AVR one year, then have to come back later for another round of OHS! Or worse, to suffer through an emergency or die from an aortic emergency.

I doubt I'll have to argue the point with the surgeon. However, I need to understand my situation, know what I want done in terms of management, and be prepared with questions.

The Emotional Response To Your Diagnosis

Finding out that I have BAV disease and an aortic aneurysm was a shock. I was already pretty well shook up after meeting with the surgeon even before this latest news. At least he prepared me somewhat when he told me that he thought I did have a BAV and that it was missed previously. I've since found out that this happens a lot with bicuspid valves because they are challenging to spot on echos.

It's normal and human to have an emotional response to this kind of news, and I'm just like anyone else in that respect. The other day I wrote to a friend who has been very supportive. I told her about our weekend trip to Florida. Some of my emotions came through in that email:

"I did my first ever snorkeling in Key Largo over 30 years ago. I was still married to my wife then. We were in Miami for some sort of convention that she was attending. She was, believe it or not, a surgical nurse and assisted in open heart surgeries! Poor woman, she used to come home a basket case because back then in the early 70s so many more patients died. Anyway, going back out on that reef once again more than 30 years after my first visit was something of an emotional homecoming for me, actually. Well, I'm more emotional these days as you can imagine, and the trip to the reef triggered a lot of strong feelings and memories. I felt like it was the other bookend to my adult life in some respects, if you know what I mean. That hour I spent out there in the crystal clear, warm water enjoying all the beautiful fish and corals was just an indescribably intense emotional experience for me. I was so relaxed as the waves gently washed over me. I hated to get back on the boat when the hour was over. I'm hoping I'll get another chance to do something similar in the future."

Stenotic BAV/Aneurysm: What Is To Be Done?

With a bicuspid valve and an aortic aneurysm, the plan seems to be to replace the valve, preserve the aortic root if it is normal (and mine is,) and repair the aortic aneurysm. The reason that it is important to preserve the aortic root (the part of the aorta that connects to the heart) is because that's where the two coronary arteries branch off. If you have to replace everything...the valve, the aortic root, and the ascending aorta, the surgeon has to reconnect the two coronary arteries to the aortic root graft. This is called a "Bentall," or " Button Bentall" procedure and it is tricky and can lead to a lot of extra complications. Once again, getting things taken care of before the BAV disease leads to aortic root problems seems like a real good idea.

So, it will be interesting to see what the surgeon says on January 6. My "numbers" are borderline, I think. According to The American College Of Cardiology/American Heart Association Guidelines :

In patients with bicuspid valves undergoing AVR because of severe AS or AR (see Sections 3.1.7 and 3.2.3.8), repair of the aortic root or replacement of the ascending aorta is indicated if the diameter of the aortic root or ascending aorta is greater than
4.5 cm.*

If patients with bicuspid valves and associated aortic root enlargement undergo AVR because of severe AS or AR (Sections 3.1.7. and 3.2.3.8.), it is recommended that repair of the aortic root or replacement of the ascending aorta be performed if the diameter of these structures is greater than 4.5 cm (366).

So mine is a complicated situation. My AS is technically still in the moderate range with a valve area of 1.3 cm2, but adjusted for my body size it falls in the "severe" range (VAI = <.6). So, by itself, my valve problem is borderline by the numbers. My symptoms do not include the most serious manifestations of AV disease (dizziness and fainting,) rather I do experience exertional dyspnea and angina.

However, I do have a BAV and an aneurysm. Surgery is recommended to repair an anuerysm if the maximum diameter is 5.0 cm or more, or 4.5 cm if valve surgery is also being performed. My aneurysm is 4.3 cm, just under the recommended size for repair during AVR. Well, it makes no sense to me to have valve replacement surgery and not correct the aneurysm. Who wants to have to undergo another OHS later on to correct an aneurysm, or worse yet, experience an emergency related to the aneurysm?

So, what will the surgeon's recommendation be on January 6? Should I wait until the aneurysm grows and/or the valve stenosis gets worse, or more likely, both problems worsen? I could do that but that doesn't make a whole lot of sense to me either. I think that the combination of moderate to severe AS AND a BAV/aneurysm will trip the "surgery now rather than later" wire.

Therefore, my hunch is that the surgeon will recommend immediate AVR and ascending aneurysm repair.

Let's see if I'm right.

Bicuspid Valve Disease

One of the best informational articles that I've found on bicuspid valve disease is here.

Here's the bottom line in a nutshell:

In general, patients with mild-to-moderate valvular dysfunction and normal left ventricular (LV) dimensions and function should be monitored by echocardiography at regular intervals. Aortic valve replacement is indicated for severe valvular dysfunction, symptomatic patients, and/or those patients with evidence of abnormal LV dimensions and function (Table). Because many of these patients will require cardiac surgery during their lifetime, early referral to a surgeon with experience in aortic valve surgery is recommended.

Basically, once someone is diagnosed with BAV, then "early referral to a surgeon with experience in aortic valve surgery is recommended."

In my case the good news is that I believe the the disease was diagnosed before it has progressed too far but only because I elected to seek out a surgeon on my own. My "numbers" are borderline severe: valve area 1.3 cm2 (technically "moderate" but "severe" - when adjusted for my body size - valve area index = .55); aortic dilation of 4.3 cm - which is in the range that can trigger surgery:

Aortic dilation should be carefully monitored by echocardiography21 and aortic root replacement recommended more aggressively for patients with BAV24 with aortic dilation (ie, 4 to 5 cm) than for those of patients with tricuspid valve (ie, 5 to 6 cm).

So, there's the scoop. I'll have my CT angio chest and see the surgeon again on January 6. To me it looks like all that is left to be done is to the set the date for surgery and select the valve prosthesis. I'll have to ask the surgeon what his plan is to deal with the aortic aneurysm.

My Valve Disease/Aneurysm Conundrum

Yesterday I went snorkeling in John Pennekamp Coral Reef State Park in Key Largo, Florida. The water was 75 degrees and waves were normal at about 1 to 2 feet. We anchored the boat about 50 yards from the edge of the reef down current. That meant that snorkelers would have to swim the 50 yards to the edge of the reef against the current. The current was not very strong, luckily. There were about two dozen people going snorkeling. They ranged in age from perhaps 11 or 12 all the way up to retirees. Since I'm a certified scuba diver with 30 years experience with a mask, snorkel, and fins, I was ready to go first in the water. I swam up to the reef then around it to the far edge, probably another 75 or 100 yards so that I could start my snorkeling there and see the entire reef. An hour later the captain blew the horn on the boat signalling snorkelers to return. I was the last one back on board. The mate told me that I covered far more of the reef than anyone else.

The nature of valve disease is such that it can cause frustration. Here I was in the water as active as anyone else on the boat, yet I have a condition that could kill me if left untreated, and will probably force me into the operating room early next year. It's a peculiar situation to live with. Basically, I can almost do anything I ever could, except for heavy exertion like running and heavy lifting. And I don't feel mortally ill. But I am.

Now added to the mix I find out that I have a bicuspid valve. Well, the literature is full of descriptions of the problems that causes. Mine is congenital, and what happens is that these malformed valves degenerate. I'm lucky to have made it to 60 with mine. Lots of people have to have surgery when they're a lot younger...in their 30s or 40s. One of the problems associated with bicuspid valve disease is aortic dilation (aneurysm,) a serious condition that is potentially fatal if not caught and treated. My aorta is dilated. So I have the double whammy: aortic valve disease plus an aortic aneurysm.

Yet, I'm still out there doing most everything including walking four miles a day, which is actually good for someone with my diagnosis. But the situation is mind boggling when you think of it.

The Big Shocker

Well, I just got back from the weekend in the beautiful Miani Beach area, and found the latest echo report from the University Of PA. hospital waiting for me.

My surgeon was right: I have a bicuspid aortic valve that the cardio missed, AND I have a dialated aorta.

And that is a horse of a different color, to say the least!

Good For The Heart!

We had a nice day yesterday here in Southern Florida. We went to the Bridge Art Fair in Miami to meet up with our friend Howard, who lives in Ft. Lauderdale. It's always nice to see friends who live in the area when you travel, and Howard graciously drove down to meet us. We had a nice lunch together. In the late afternoon we spent some time around the luxurious pool at the Fountainebleau. It's fun to live the life of the rich and famous, if even only for a weekend! We spent the evening in South Beach. We had dinner in a fine Japanese restaurant that offered some of our favorite sake and foods. They went went for a stroll on Ocean Drive, which was busy as always. If I had a choice of places to have a getaway apartment in Florida, South Beach would be my pick hands down. I don't care for Ft. Lauderdale. It's boring and there's nothing to do there. South Beach is always fun!

Weekend Break

We're on a short holiday to Miami Beach. The weather is warm and we're staying at the beautiful newly refurbished Fountainebleau Hotel.

Sleeping With Heart Valve Disease

Sometimes sleeping at night gets uncomfortable with palpitations and lately a dull uncomfortable feeling in the chest. Luckily I don't get short of breath, but I can feel the problem. Most of the time sleeping on my right side is the most comnfrotable position.

Good Advice On Selection Of A Surgeon

In response to a question of mine on VR.com, here's some great advice from Al, another member of the forum:

"There are several good reasons to start the Surgeon Selection Process early:

1 - To avoid having to select a surgeon from a gurney in an ER somewhere which will greatly limit your choices, i.e. to some surgeon who operates at whatever hospital you landed at and who has an opening in his schedule.

2 - You may want to interview more than one surgeon to find someone you 'feel comfortable with', i.e., so that you are 'prepared' when the time comes.

3 - To have the Timing and Type of Surgery defined by the guy who will actually be performing the operation. The job of your Cardiologist is to make a diagnosis and recommend a treatment plan. Medications can 'postpone' surgery (but may allow damage to progress). Only a Surgeon can FIX a valve problem (i.e. Repair or Replace a defective valve). IMO, Surgeons have a better perspective on WHEN to proceed for the Best Outcome.

4 - It would not be out-of-order for you to ask your Cardiologist for his recommendation of surgeon(s) for you to interview. Just tell him you want to "Be Prepared" beforehand. If he resists, you can do your own search. You will need copies of your Echocardiogram and Angiogram (Cath) Test Reports AND Films / CD's / DVD's. Doctors and Hospitals are required to provide those to patients by law. They may charge a copying fee, but usually waive such charges if it is going to another Doctor. You will need to sign a "Medical Release form"."

The Delayed Echocardiogram Report

I'm still waiting to get my latest echo report. I called the surgeon's office yesterday and they told me that they just received it. Really? Since November 20? Where has it been all that time, I wonder? What if it had contained some dire warning...an aneurysm about to burst? I could be dead my now. Anyway, a nurse has to review it then they'll send it out to me, I was told. I guess they have their policies but this all seems peculiar.

I'm enjoying my walks still and yesterday afternoon I walked my two miles at a fairly quick pace. I am breathing harder than I should have to but except for that I feel pretty good. There's the rub. If I'm told the time has come for surgery and I'm still feeling pretty good that makes it tough in some ways. It would be easier to go into surgery if I had severe symptoms, but that would mean damage had already been done to the heart. So I'll listen to the recommendation of the surgeon when i see him on January 6.

Symptoms And Timing

In regard to heart valve disease, there are symptoms and there are SYMPTOMS. The signs I have I've ignored for four years are difficulty with hills and steps, occasionally getting winded, palpitations, etc. Last year I mentioned some of this stuff to my cardio and he responded "you're 58, you're getting older, and you've put on 20 pounds." Well, I figured, I guess I'm guilty! Seriously though, I don't get dizzy and I have not fainted, which are the classic "severe" category symptoms that lead directly to the OR.

So, is it time for surgery?

Here's one fellow's story as posted on heartvalvereplacement.com:

I had a murmur detected at about 13 years old. I remember AORTIC REGURG. being used as well as slightly enlarged. Then I was told "SOMEDAY" I would need AVR.That was 30 years ago. FF to April 08 when I was NOT OK'd for a police job because of SEVERE AS/AR. An echo said the area was 1,2cm2 but could be less. A cath said it was really .8-.9cm and I was handed a card for a surgeon and told I have an appointment. SOMEDAY was 07-09-08. I thought I was not symptomatic but I guess I really was. I'm NOT in that bad of shape to be "HUFFING AND PUFFING" on the hills I was walking up. Heck, I'm only 43! I had an ON-X Aortic Valve put in and, while I am not at any ball games, I do feel LOTS better. I started back to work yesterday and although I am not out chasing bad guys, I am back. I feel a bit more normal each day. It won't be long till I an 110%. That's about it. Don'twait too long. I hear that the best results come before patients are symptomatic.

A Strenuous Swim Triggers Exertional Angina

It was a beautiful late August summer day. The ocean was warm and the crystal clear Gulf Stream current was close in to shore. You could see your toes standing in shoulder deep water. It wasn't very windy and the wave action was light. Often times with conditions like these you can see tropical fish from far away waters carried North to Long Island by the currents.

I decided to swim out to the sand bar with my scuba mask and snorkel on. The swim out was slow and easy. There were jellyfish in the water but I could see them and avoid being stung. I got the sand bar and stood there briefly before heading back toward the beach. On the way back a couple of larger waves broke over me and flooded my snorkel. I cleared the snorkel and continued swimming but I began to get a little panicky, fearing that I would be unable to make it back to shore. My heart started racing. I felt the sting of a jellyfish brushing my arm. I began swimming harder, harder than I was used to.

Finally, I made it back to shore, but I was tired, out of breath, and I had chest tightness which extended into my left shoulder. I had angina. Rather than sit down and rest I thought I would feel better "walking it off." So I put Bradley on his leash and took a slow walk down the beach. After about 45 minutes the angina went away. But for the first time I realized that I now had significant physical limitations as a result of my aortic valve condition. The level of exertion that reached during my swim back to the beach had triggered angina. People with moderate to severe AS should not exercise at that level.

AVD: Genesis - Progression - Angina - Palpitations

While things are intense for me just now, with the possibility of surgery looming, seeing the surgeon, and going for specialized tests, I can track the progression of my heart valve disease over decades. It didn't just happen. Rather, it actually started when I was born.

I knew even as a child that I had a heart murmur. No one seemed to think it was serious and no one ever recommended that it might be a good idea to keep an eye on my heart as I grew older. The other day I remembered going to a doctor maybe 15 years ago and complaining about the pounding in my chest. He didn't think there was anything wrong with me, and maybe there wasn't at the time. He just advised me to drink decaffeinated beverages. He didn't recommend any heart monitoring. Perhaps he should have.

When I found out in 2004 that I have AVD, I was still jogging. I remember I could jog for 20 or 25 minutes and then finish off with a sprint during the final minute. Even after I got the diagnosis I could still jog, and did so three times a week to prove to myself that I was all right in spite of the AVD. But by 2005 I found that I couldn't jog anymore. When I tried jogging I got an uncomfortable feeling in my upper chest - lower throat area, a tightness that felt like my head was going to blow off.

I saw a different cardio in January of 2006 for a second opinion, and I told him about this. We did a nuclear treadmill stress test then, but it was negative...at least the cardio made no diagnosis based upon the results of that test. My AS was still technically "mild" at that time, in all probability by the numbers, so he was probably looking for any evidence of CAD. However, I think the sensation I was experiencing at that time was related to my AVD. I may have been further along with the AS adjusted for my size even then, and he missed it.

So I now believe that this strange uncomfortable feeling, this tightness that I get is angina related to my AS. And I've been experiencing it since 2006. Angina, "chest pain," comes in many different flavors. It's not simply a sharp or dull pain. On of the best descriptions of angina I've ever read was posted by a fellow named Bob on the Heartvalvereplacement.com website with emphasis added by me:

Many people have angina and don't realize it. The classic is the left arm, left shoulder/back, or left-side-of-chest pain. However, it can also show up as a feeling at the top of your lungs like you've been breathing cold air, or a feeling like a lump in your throat, a tightness across the chest, or just a slight throat tightness. It can show up solely in your back, or be confused with heartburn issues. Or you can feel it as neck or jaw pain (this is especially common in women).It may well show up when (or just after) walking uphill or up stairs, although not necessarily every time you do it. It's often not the dramatic, agonizing pain that is portrayed on TV shows, but more of a lower level discomfort or an uncomfortable tightness. It can also show up at the end of the day, with you becoming aware of it when you first lie down (especially if you flop down after climbing stairs). You may also become aware of palpitations, which are misbeats or arrhythmias, in the evening when you are quiet. They tend to get worse as you approach surgery, and visit you in the darkness at night, when your spouse is asleep, and you are alone with the shadows on the ceiling. They tend to bring some sense of panic with them, which makes you more aware of them,and can generate a bad, endless-loop cycle when you most need sleep.

This is the most comprehensive description of angina that I have ever seen. Bob has it just exactly right. His comments on palpitations are spot on also. The bottom line is that I've had angina for a couple of years now, and palpitations for may years that have gotten worse particularly over the past year. During the past year, the palpitations have become so strong at night that I can't lie in bed on my left side or on my back for long. I have to sleep on my right side only.

When you put this all together, symptoms for two years or more now, severe AS, adjusted for body size...well, I didn't get to the surgeon a minute too soon, it appears. It will be interesting to see what he recommends on January 6.

The Valve Area Index Calculation

One factor that has to be considered in assessing valve disease is your physical size. I found this out when I met with my surgeon in November. He told me that while my "numbers" fell within the guidelines for "moderate" AS, since I am "a big guy" that meant that my AS might already be severe. At one point during our meeting he sat down and gave some numbers off the top of his head to the RNP working with him and asked her to do some calculations. I didn't understand at the time, but I now believe he was doing valve area index (VAI) calculations.

Your VAI is your valve area in cm2 divided by your body surface area. In my case, as of my echo in August my AVA was calculated at between 1.1 and 1.4 cm2. My current body surface area is 2.35 m2. Plugging these values into the formula I come up with a VAI of between .468 and .595. According to the guidelines, anything less than 0.6 indicates severe AS. Even if I was at my ideal weight of about 200 (I'm 226 now), my VAI would still be severe or borderline severe at between .49 and .625. I'm a little fuzzy on what the surgeon said exactly, but t was something like they want to do the surgery when the number goes below .5.

Where is that latest echo report copy from November 20th? I sure hope I get it this week.