Saturday, January 3, 2015

Aneurysm Surgery - How Much Aorta Was Left Behind?

Another Controversy

It was hard to believe that my husband had such a major surgery. Of course that wound down his chest, along the same scar line as his first surgery, proved that his chest indeed had been opened again. 

The procedure was Wednesday morning, by Friday he was asking to go home, and Saturday morning he was discharged! Amazing - the third morning post op! He healed and recovered well.

But what had actually been done inside his chest? Would it stand the test of time? Later I would learn that there are things about aortic surgery that are debated too. I mentioned previously that there are two controversies that my husband escaped. One was the size of the aneurysm when surgery should be done. Here I write about the second one: whether or not to remove the entire ascending aorta using total circulatory arrest. Today I am so thankful that, in the right hands, my husband's entire ascending aorta was removed!

23 Minutes of Circulatory Arrest

The surgeon spoke to us after my husband's surgery, before I went in to ICU to see him. I wrote down something about 23 minutes, but at the time I did not really understand what it meant.

Later I would learn more about this. I would realize that my beloved's head was packed in ice during this time. Extra protection during a time when his brain waves went to zero! Nothing, no brain activity at all! I would eventually learn something about the history of this. And one day, I would go into surgery and watch it done to someone else's husband.

How Much Aorta Was Left Behind?

Perhaps there are many debates about the exact "how" of aortic surgery. This one is particularly meaningful and important to me.

The largest "bulge" of the aneurysm for those with BAV is usually in  the middle of the ascending aorta. Of course, there are exceptions to this, as there are exceptions in all things BAV!

The simplest, easiest surgery is just to cut out that piece of the aorta that is obviously bulging, leaving behind the part of the ascending aorta closest to the arch (the curve of the "candy cane") or so-called top part of the ascending aorta.

Why is this simpler, easier? Because the surgeon simply puts a clamp on the top of the ascending aorta, allowing the heart lung machine to keep circulating blood while cutting away the aortic tissue lower down.

Very Cold, No Blood Flow ( Deep Hypothermic Total Circulatory Arrest)

But what if the surgeon removes all of the ascending aorta, right up to the arch? There is no place to put a clamp. How can this be done, if the aorta is wide open, without blood pouring out of that opening?

Well, the flow of blood has to temporarily be stopped (circulatory arrest). That can be done by stopping the heart lung machine, but how can a patient survive that? Every cell in their body needs proper blood flow.

People can survive temporary blood flow interruption if they are kept very cold. The term used is deep hypothermic total circulatory arrest. As you can imagine, this is not easy to do without injuring the person whose life you are trying to save from the aneurysm.

My husband had this procedure. This was the 23 minutes, when his brain waves went to zero, he was very cold, and no blood circulated in his body. Yes, his head was packed in ice for extra brain protection. Unaware of all this, I sat in the waiting area. I was waiting to hear those wonderful words "the aneurysm is removed." At last, I heard them, some of the most special words ever spoken to me! A tremendous feeling of relief flowed through me at that moment. I remember that feeling, that moment to this day. Those words, that feeling, are tucked away among the special "sounds" in my heart.

When someone was talking to me about this part of surgery, they called this the part of the operation "where they deaded me"! We might think this sounds like death, and it would be at room temperature perhaps. But when very cold, the brain is protected, and comes back beautifully when slowly re-warmed.  This is what prevents brain and other organ damage to those who fall through the ice in frigid northern climates. They come out just fine, if they are rescued from the icy waters in a reasonable time.

Every time I think of my husband's aorta, I am very thankful that his entire ascending aorta is all gone! There is actually a "tongue" of Dacron underneath his arch, as his tissue there was not "good". So, it is a very good thing for him that the hands he trusted had the skill to do what was needed and nothing was left behind.

Not All Surgeons Do Circulatory Arrest and Take the Entire Ascending Aorta

There is a man, still young today, who lives in a rural setting. I picture him and his family enjoying those wonderful, wide open spaces. He does not live near any major medical center. As a young husband and father, he had his BAV and ascending aneurysm replaced. He and his wife traveled to a major medical center, far from home, for this surgery.

I well remember asking them how many minutes he had been under circulatory arrest. I wondered how long it had taken. My understanding was that up to 30 minutes was generally safe. Much to my surprise, they were told that it had not been necessary to do it, that the upper part of his ascending aorta was "fine". The years have passed. What about the aorta that was left behind? What does it mean for him? It is something to be watched. I think of him and his family often.

Since then, I have heard of many surgeries where some part of the upper ascending aorta was left behind. In that moment, done by those hands, perhaps it was the safest that could be done. But what about these people in the long term?

Thankfulness but Not Forgetfulness

Today my husband and I are grateful for life-extending surgery. Without it, his own and so many precious lives would end much too soon. But none of us can forget about aortic disease, once it enters our lives. There are things we can understand to help us live more confidently, although we may have aortic disease.

Before Aneurysm Surgery

I was given some good advice about surgery once. I was told never to urge a surgeon to do a specific technique on me, in order to have me as a patient. Surgeons will offer their patients what they do best. Just listen and try to understand. I was told not to push them for something else. If I did, I might be the first patient they try it on! Especially with something as complicated as what is described here, if the surgeon did not offer it to me, I would never ask for it. I would continue my search for help.

If a surgeon did offer it to me, I would ask how many of this exact procedure his hands do on a yearly basis. I would also ask how many complications (bleeding, stroke) there were. I would ask how many deaths. It is important to ask these questions.  It is my life, my future at stake.

I would also try to find someone who works at the hospital, who knows this surgeon's work. I am thinking of a woman who did this. She found out that the number of procedures that the surgeon quoted her was not for the heart procedure she needed at all. She had been given confusing, if not outright misleading, information. Being both brave and intelligent, she continued her research and ultimately traveled out of state, where expert hands performed this technique during her surgery.

After Aneurysm Surgery

If I already had my aneurysm removed, I would try to learn how much of the ascending aorta was left behind. One way to do this is to get my surgery report from the hospital medical records, and read it. Usually the surgeon's report describes in detail what was done.

And then, every few years, I would have my aorta scanned. It is better that I leave nothing to chance. It is best to know if my aorta has changed or not, since my surgery and since the last scan I had.

Time Tests the Surgeon's Work

Yes, surgeons may continue to debate the best techniques in aortic surgery for years to come. While they are debating, men, women, even children live with their work inside their chests. Time tests each surgeon's work.

I am very grateful that this is one debate that does not touch us. His entire ascending aorta is all gone. It cannot hurt him now. Something else did hurt him. It hurt him very badly. I will share that next.

May all with aneurysms be kept safe,
 both before and after their surgery,
                - Arlys Velebir

Aneurysm Surgery - When to Have It

Diseased aorta, including ascending aneurysm
Avoiding the Controversies

I will always be thankful that there are two controversial issues in aortic surgery that can never hurt my husband. (He has found enough complications on his own, without anything more!)

One of those controversies is when to have surgery!

It may surprise you that there is a great deal of debate among physicians about some things that are often said to patients with great confidence, as if there were no questions at all. 

One of them is the size of the aorta that justifies surgery.

Two Men, Real Life

I will never forget Richard. He is close in age to my husband.  His aortic aneurysm was close in size to my husband's too.

I held Richard's hand the very first time I met him. I held it to comfort him. He was a patient in ICU that day. He was a miracle to me then. He still is. Richard is a survivor of aortic dissection.
Richard Houchin (in blue) running again
 after surviving aortic dissection

Why did one man's aorta tear,
 and not the other?

Why did Richard's aorta tear,
 nearly killing him,
 and my husband's did not?

No one can answer that question.

Clearly, it was more than just the size
 of the aneurysm.

When to Have Aneurysm Surgery

When it was discovered, my husband's aneurysm was measured at 5.2 cm at the widest part. He had an echo first, than a CT, and also an MRI. How we hoped there had been some mistake, or that it would somehow shrink. There was no mistake. And aneurysms do not shrink.

We had read before we met Dr. Raissi that aortic surgery had been done at 6.0 cm historically, when the surgery was still quite risky. As the surgery became safer, it was being done at smaller sizes. Based on what we had read, we believed my husband's aneurysm qualified for surgery in properly skilled hands.

One of the papers I read at that time, from Mt. Sinai in New York, spoke about when and how aortic surgery should be done. The paper includes those born with BAV.  I was very grateful for this paper, presented at the Aortic Surgery Symposium in 1998 and then published:  Surgical treatment of the dilated ascending aorta: when and how?  I remain truly grateful for Dr. Griepp and all the great work done at Mt. Sinai guided by his vision, skill, and compassion.

We were glad that when we found our way to Dr. Raissi, he did not hesitate to offer aneurysm surgery to my husband. Neither did he immediately rush him into the operating room. He did treat his blood pressure immediately, adding medications and adjusting dosage until it was well under control. During this time, we chose a surgery date. How was his surgery and recovery? Everything went perfectly. My husband's aorta remains in excellent condition, time tested and monitored for almost 14 years now! (His valve experience is a different story, which will be told later!)

Sadly, despite continued discussion over the years since my husband's surgery, today there remains a great deal of conflicting thinking about when an aortic aneurysm should be replaced in someone with BAV.

What's the Clinician to Do?

In a recent editorial in Current Opinion in Cardiology, Dr. Alan Braverman discusses the variations in recommendation in recent guidelines: Guidelines for management of bicuspid aortic valve aneurysms: what's the clinician to do?

Notice the differences in the guidelines in the chart.

Dr. Braverman writes: "I do not believe 'one size fits all' when it comes to decision making about the timing of aortic aneurysm surgery. One has to individualize management based on each patient's characteristics."

Yes! This is comprehensive, individualized care! This is what we need!

He further writes, "However, I am concerned that for some bicuspid aortic valve patients the strategy of observing the dilated aorta (greater than 5 cm) while waiting for the diameter to exceed 5.5 cm before recommending surgery will be associated with an unacceptable risk of aortic dissection."

However well meant, confident statements about waiting for surgery until a specific aneurysm size is reached, implying safety to the patient until then,  may be followed by tragedy for some people. People who believed they were safe until the moment their aorta ripped inside them. These are the people exposed to unacceptable risk when told to wait for a certain size aneurysm to develop.

What about the risk of the surgery? To me, this is a moral issue. Complex aortic surgery will be safest in the most skilled hands. These are the hands I would want if I were to have this surgery.

The moral issue is that patients need to have surgery in those most skilled hands, but may not be helped to reach them.

I personally would not want to have my aorta tear while waiting for a larger size aneurysm because I may be injured or die from the surgery. I am being forced to wait because the surgeon may hurt me before the aneurysm will!

I would feel I am being asked to gamble, trying to have as many good, quality days of life as possible before one or the other ( the aneurysm or the surgeon) injures me or worse!

There are highly skilled aortic surgeons. I should be able to go to one of them. I should not have to gamble with my life.

Dr. Braverman concludes his article with: "In discussions with cardiologists and surgeons who have expertise in managing the aortopathy of bicuspid aortic valve disease, it is my impression that they will use many factors other than size alone to inform the decision as to when to go forward with aortic surgery. We all should!"
Well said, Dr. Braverman!

What's the Patient To Do?

This debate and uncertainty makes it very difficult for someone born with BAV who has an aneurysm. We are expecting trustworthy advice about when to have surgery that is based on solid knowledge, with the assurance nothing "bad" is going to happen to us until then.

After all, what is the point of knowing you have an aneurysm, living each day in the shadow of that knowledge, only to suffer injury or death from it?

We need to know what is known.

We need to know what is NOT known.

We need to know and be part of the decision making process. Knowing so many with BAV and their families, I believe we can handle the truth.

So, what is the patient to do? Find someone who will tell us everything.

Find someone who will tell us all about what is known about size. Tell us that the larger the aneurysm, the riskier it is. But tell us that smaller sizes are not safety guarantees! Tell us that some aneurysms tear at sizes smaller than 5.5 cm. My friend Richard certainly knows that!  Tell us that some aneurysms tear at sizes smaller than 5.0 cm too!

And then tell us more.

Tell us, as Dr. Braverman describes, all the factors, the pros and cons, that apply to us as an individual.

Help us decide, thinking about many things and not just size alone, when to have surgery.

We need to find the hands that are expert in aortic surgery, with the best possible outcomes. Will someone tell us who they are? We are on the brink of greater transparency in medicine in the US. My hope is that will help us, the patients, search out the right hands.

We are the ones that live each day with an aneurysm in our chests. The risk is ultimately ours.

Please, tell us everything.

We can handle it.

Just don't let us die
 without the best possible chance
 to win this aneurysm fight. 
I believe this is what my husband has had. 
I wish the same for everyone.
                                   - Arlys Velebir

Thursday, January 1, 2015

BAV and Aneurysm - The Search for Help

"If you cannot do great things, do small things in a great way."  - Napoleon Hill

The Search for Help
Diseased aorta, including ascending aortic aneurysm

bicuspid aortic valve ascending aortic aneurysm
That is what I typed in the search engine in 2001. I found important answers from a somewhat surprising source. Williamsport, Pennsylvania, is a town of about 30,000 people. It is not the home of any famous heart centers. I am not aware of any well known heart specialists who practice there.  There would not be any large research grants or costly, multi-year studies of patients happening there. But in 1998, cardiologists there published a paper that is very important. In 2001, it was this paper that helped us understand what no physician had explained to us: that someone born with BAV could have an aneurysm, and get into trouble, whether their valve has been replaced or not. Someone like my husband. After his successful surgery, I wrote to Dr. John Burks to request the full paper and thank him. I am forever grateful and will treasure this paper always. Just one paper, perhaps it may seem small, but it was done in a great way. I like to think it awakened others and spurred them to do more for those born with BAV. 

Gone Fishing

Here I will spend a moment on our local experience. Once the CT confirmed the aneurysm finding of the echo, our internist suggested we contact the surgeon who had replaced the valve about 11 years earlier. Although we continued to search online, I did go ahead and call the surgeon's office. I remember telling the woman I spoke with that my husband had an aneurysm. I am not sure if she was a nurse or not, but there was a serious tone in her voice after she heard me say those words. She told me that the surgeon was out of town, on a fishing trip with the cardiologist that we also had met 11 years earlier.

This was a good thing.

We did not waste any further time with them. Today I know that there is nothing in their backgrounds that would particularly equip them to handle what some call the most difficult surgical procedure in the chest: aortic surgery. Years later, someone locally told me that this same cardiologist first referred them (adult man) to a surgeon whose specialization was in pediatric heart surgery! Then they were given another name, in Houston, of an aortic surgeon who had been dead many years. I suppose I was not surprised, but I was certainly saddened, at such inappropriate "help" being suggested.

Still today, I rather marvel that the internist did not have more knowledge or take more time with us. He was in many ways a very kind man, but clearly not equipped in the face of BAV and aortic disease. Kindness alone cannot save lives.

Regardless, it was time to move on. We were on our own.

Searching online, I discovered PubMed, the online US National Library of Medicine. What a treasure! I read that the aorta is replaced by a "tube" made of Dacron. I read about the aneurysm sizes at which the aorta is replaced. I tried out a calculation based on my husband's height and weight versus aneurysm size. Most of all, I searched for surgeons who did aortic aneurysm surgery in the chest.

Meant to Be

Did we really know how to look for help? Not very well, but we could follow clues. Today, when I think back to that time and all that has followed, the phrase "it was meant to be" comes to mind. We did not know then so many things, that eventually we would learn.

We did not know about the pioneering work of Dr. Randall Griepp in New York, and his collaboration with Dr. Nicholas Kouchoukos in St. Louis, among others. We did not know that Dr. Griepp had mentored Dr. Sharo Raissi in aortic surgery, and that Dr. Raissi had brought his exceptional aortic surgery skills quite near to us.

At a time when the bulging aorta, and worse yet, the horrendous bleeding emergency of aortic dissection, were viewed by many physicians with helplessness and dread, these skilled, courageous, and compassionate surgeons ran not away from the fight, but directly into it. They are the "special forces" of heart surgery - the most skilled, the bravest, the best. The biggest challenge yet today for those with BAV and aneurysm is getting themselves to skilled hands like these.

Perhaps some angels paved the way to Dr.Raissi's office that day. We were rather terrified really, consulting a surgeon on our own after being told years before "never ask a surgeon if you need surgery"!  The aneurysm terrified us more.

About half way through that appointment with Dr. Raissi, I felt the icy fear begin to melt. We went home with blood pressure medication prescriptions and strict instructions to start them that very night! Wonderful! At last we had found someone who understood and cared.

Regardless of how it came to be, we know how hard it can be still today to find help. We share our story to help others who also must travel this road after us.

Here is a video where I describe our search and aneurysm surgery experience.

 I also mention here that this aneurysm surgery was not the end. My husband would need another valve surgery just 5 years later, in 2006. Perhaps it has taken all these years, and we are still learning today, the reality that some born with BAV are never "done"!

Best wishes to all who read this, in this New Year of 2015. May you have happiness, good health, and be led to those who can help you in your time of need.

                                                                                       - Arlys Velebir