The Aortic Valve - What Can Leaflets Tell Us?

Da Vinci's sketch of the aortic valve, including BAV

Five Hundred Years Ago

About 500 years ago, Leonardo da Vinci studied human anatomy. From his access to animals and also human cadavers, he explored the mysteries of life through the dead. Outstanding still today among his amazing work are his studies of the heart and its valves, including a sketch of aortic valves with abnormal leaflets, including those with only two leaflets (bicuspid) and four leaflets (quadricuspid). 

This aortic valve was consistently called a normal three
leaflet valve when viewed by echocardiogram. After removal,

it is clear that two of the leaflets are fused. The man born with
this valve had no reason to think about his health or his heart
until the day searing pain dropped him to the ground.
His aorta had dissected.  Picture courtesy of Dr. Sharo Raissi

Where We Are Today

Despite technology that shows us inside the living beating heart today, we still struggle to clearly and accurately "see" the aortic valve during life and to understand what it means.



Why is it so important to see clearly? The entire picture is needed to help someone. Abnormal aortic valves are associated with other problems, including infection (endocarditis), abnormal aortic tissue, aneurysm. and dissection.



The valve pictured here was consistently read as trileaflet by echocardiography. Only when it was removed did the fusion between two leaflets reveal itself to the world. An aortic valve with three leaflets is said to have a triangular opening, a bicuspid valve to have an oval opening. This valve has a triangular opening, although there is fusion of two of the leaflets. Someone with a valve like this may have an echo at some point in life,  be told their valve is normal, and never be monitored for the development of an aortic aneurysm.  They are vulnerable to an aortic emergency, like the dissection that struck the man whose valve we see here.

The Importance of Leaflets

The Bicuspid Aortic Foundation uses wording like "apparently normal" or "appear to be normal" to describe aortic valves with three leaflets in the presence of aortic aneurysm and dissection or rupture. This is because we know that there may be something wrong with these seemingly normal aortic valves and their leaflets. It is just more subtle. Bicuspid aortic valves have been associated with aortic aneurysm and dissection for a very long time. What about those three leaflet aortic valves out there? It is not possible to take a snippet of tissue and study it, but is there a way to find slight abnormalities through imaging tests?

Warning for BAV Families in 2007

In 2007, I read about 13 families in this paper, Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance. These families all had at least one person with a BAV. But there were others in these families who appeared to have normal aortic valves with all three leaflets, yet they still had an aneurysm. Some of them dissected and died. I knew it was important to tell BAV families about this, to help save the lives of everyone in the family. From that time, it was clear that just checking the aortic valve was not enough to keep family members safe. But the question has remained, are these apparently normal trileaflet valves really normal, or just part of a continuum of BAV?

It's 2015, and Dr. Sperling's Paper Sheds New Light

The recently published work of Dr. Jason Sperling, a member of the BAF Scientific Advisory Board, has shed new light on aortic valve abnormalities in this paper: "Forme fruste or ‘Incomplete’ bicuspid aortic valves with very smallraphes: The prevalence of bicuspid valve and its significance may be underestimated".  Pictured in this paper are aortic valves with very slight fusion of the leaflets, in the "corners" where they come together. These are aortic valves whose abnormalities are so slight they are easily missed. Dr. Sperling's work shows how these slight abnormalities may be found through imaging.

There is no doubt that the more we understand about the mysteries of the aortic valve, we also understand more about the big picture of aortic disease in our bodies.

Thank you so much, Dr. Jason Sperling,

 for your careful attention to detail and 

your dedication to unlocking

 the mysteries of the aortic valve.

You are creating a climate of hope

for patients everywhere.

- Arlys Velebir

                           BAF President and Chairman

The Heart of a Man - Angelo's BAV Diagnosis

"I am writing to you because I simply don’t know where else to turn." 

On any given day, somewhere in the world, a man born with a bicuspid aortic valve learns about it for the first time. 

Why a man? Because BAV is up to 3 times more common in men than women. What is it like for this man, typically healthy, with little need for visits to doctors' offices? 

Angelo and his wife, Australians,  visited with some of us in southern California
Arlys Velebir, Mary Houchin, Richard Houchin, Angelo, Marie, Dr. Sharo Raissi

Here we share one man's experience. Angelo Doukas has given us permission to share the letters he wrote to the Bicuspid Aortic Foundation shortly after his diagnosis. We have removed a few personal details and added subtitles, but the following, mainly untouched,  is Angelo's beautifully written account of his experience .

First Ever, Routine Heart Check

Just over one week ago, a routine heart check (the first I have ever done in my life) revealed that I have a bicuspid aortic valve and aneurysm of the ascending aorta.

My cardiologist, who I had just met, explained what this meant and showed me the ultrasound images.  He explained that a normal aorta is about 3.5 cm and that when my aneurism grows from its current 4.7 cm to 5 cm, surgery will be required.  He explained how the aortic valve is supposed to operate and how mine does, how the condition has familial links, and so on.  He asked to see me again in six months.

     

I think I will always remember his parting words for the rest of my life:  “nothing will happen to you in the immediate future, but it’s a problem”.  I’m not used to hearing those words.

Shock

I sat in my car afterwards in a daze, not quite sure what this was all about but knowing that it was serious.  I was in shock, but not yet emotional because I didn’t know enough about the condition to form any opinions.  

I came home, started researching on the internet and came across your website.  I read about the condition itself (I still can’t bring myself to use the term “disease”) and then read your stories of hope.  That’s when I became emotional. 

In a single day, my life completely changed.  Until that day, I was a healthy 55 year old who always looked after himself.  I am a vegan, I exercise, have never smoked, have very little stress in my life and a wonderful, supportive family.  My parents are still alive and well, and I looked forward to a healthy old age.

My Life Will Go On!

One week later, I am still emotional, but my focus has changed.  Your website and a follow up discussion with my family doctor have reassured me that my condition does not mean an end to my life.  It will never be the same, but it will not end.  If nothing else, I’m grateful that I’ve reached this age without symptoms and without requiring surgery.  I’m also grateful I discovered the condition at 55 years of age.  I have enough time to mentally prepare for the challenges ahead, and I have lived my life until this point in time without the burden of knowing what lay ahead!     

A Father's Heart

But as I said, my focus has changed.  This week I’ve been thinking of my two children, both of whom are in their late twenties.  My cardiologist (I am also struggling to say “my cardiologist”) has strongly recommended that my children are checked.  I know this is the right thing to do and have told them they should do it.  But I live in fear that one or both of them have this condition.  Just the thought of them having to undergo a future procedure is enough to weaken me at the knees. 

When I broke the news to my family doctor he looked me in the eye and said “I would much rather be telling you that you had this condition rather than cancer,  or a neurological condition, or an autoimmune disease”.  His words were comforting at the time, and remain so, but I can’t help but reflect on the fact that none of these conditions necessarily involves the rest of the family.

You have mentioned that one of the worst things about BAV is that it’s a silent killer because healthy people are blissfully unaware they have it.  I would argue that the worst thing is the familial link because of the guilt and emotions and repercussions that result from it. Your web site talks a lot about frail aortas, but right now I am feeling so emotionally frail thinking about my children that I often break into tears.

Going Back for More Information

Now that I am much better informed, I have booked another appointment with my cardiologist for early next week (there was no way I was going to wait six months!) to have some of my questions answered.   I will also take my wife with me, because I would like her to hear everything first hand.

After the Second Cardiologist Visit 

 He (cardiologist) had asked me to come back in six months’ time, so he was very surprised to see me.  I had booked the appointment without him knowing!  I think he was genuinely concerned that he had created so much anxiety in me, so he gave me as much time as I wanted.

I feel so much better after this second visit, for two reasons.  Firstly, it appears that my bicuspid aortic valve is in excellent shape and, at 55 years of age, is still performing as well as a normal tricuspid valve (no abnormal leakage, etc.) and could continue to do so indefinitely.  My wife can verify his exact words: “if the rest of your body was up to it, you could compete in the Olympic Games”.

Angelo chatting with Dr. Sharo Raissi
in southern California

 Although I have no intention or desire to compete in the Olympic Games, it was music to my ears.  He reminded me that he had made that point in our first session and, frankly, I believe he had.  But so many arrows were being thrown at me from all directions that I was only hearing the bad news.

Secondly, my aneurysm is 4.6 cm, not 4.7 cm.  Not a big difference, but I’ll take every millimetre I can get.  He mentioned that he had many patients (one of them was actually in the waiting room) who stayed on this size for many years.

In summary, your lovely letter, in combination with this second visit, has put me in a much better state of mind.  My fear about requiring imminent surgery has subsided, and I am more confident that surgery, at least initially, would only be required for the aneurysm and not the valve.

Believe me, I am not being complacent, nor am I taking anything for granted.  But as you so correctly pointed out, at least now I know a little more about what’s inside me, and I can work with my cardiologist to monitor the problem and act on it at the appropriate time.

My only remaining concern relates to my children.  I have advised both of them to have a test and, at least for the foreseeable future, I will now leave it up to them.

Thank you so much, Angelo, for allowing us to share this. I am sure it will help many  who are learning for the first time that they were born with BAV. 

                                                                                 Best wishes,

                                                                                 Arlys Velebir

                                                                                 BAF President

Bicuspid Aortic Valve and Aneurysms - Ten Years Later

Doug in September 2004

Ten Years Ago, in 2004

Ten years ago, on September 20, 2004, Doug Grieshop died when his aorta ruptured. He had an undiagnosed bicuspid aortic valve and an aortic aneurysm in his chest.

Eighteen months earlier, Doug went to the ER with chest pain. Nothing was found then. It was left for the coroner, after his death, to find the scar of an old tear, next to the rupture site.

A weakened, diseased aorta may "hang on" for some time, because it has an outer layer designed to be stronger than the others. For Doug, that outer layer held on for a year and a half. When it broke, there was no time to save him. He was instantly gone.

What would happen ten years later, in 2014?

If Doug went to the ER, would they still check him for a heart attack, find he did not have one, and stop there?

Or would they go further? Would they find his bicuspid aortic valve, his aorta bulging dangerously?

Would today's technology be able to "see" the small tear in his aorta? If not, would they still take his chest pain and aneurysm seriously?

Would they ask him about his family history? His uncle died in the same way. Family who saw them say that in death they both looked like they had "exploded". A horrifying memory.

Would they refer him for surgery in time?

 Ten years later, would someone save Doug?

In some communities in the United States, we know what happens today. Some aortic emergencies are being found in time to save lives. But we hear of the dying. Still. In 2014.

For one man, it happened like this.

Always healthy and active, he arrives in the ER. He tells them about a feeling of pressure and burning in his chest. He is short of breath. They check for a heart attack. Negative.

They know there is another killer in the chest - pulmonary embolism (blood clot in the lung). They rule that out too.

They keep him in the hospital, continuing to speculate. The family hears words like acid reflux/heart burn. They even hear about sleep apnea! Eventually they hear he has a bicuspid aortic valve that might need surgery "some day".

Although BAV is a red flag for aneurysm in the chest, finding it did not raise the alarm. Was it because there is a persistent impression that bicuspid aortic valves are relatively harmless?

For this man and his family, many hours went by, spent guessing about conditions that are not immediately deadly.

At times, he felt better. But all of a sudden, something terrible happened inside. At that moment, it was already too late. Many hours were spent desperately trying to save him. It was impossible. Time had run out.

He had bled massively inside his chest. The respirator would continue to breathe for him. His brain was dead.

Is this the best we can do, 10 long years later?

There are many unanswered questions and conflicting guidelines for those with bicuspid aortic valve.

We know we need more answers.

However, we ask for those with BAV what we ask for everyone else. We ask that this often varied condition be viewed with respect for its potential deadliness.

Please do not forget that the diseased aorta is right up there with heart attack and pulmonary embolism.

It is a killer.

There are little children growing up without Daddy,

 grieving wives, heart broken parents.

 Brothers and sisters wondering if this will strike them also.

For everyone with a bicuspid aortic valve

 who will never have an aortic emergency,

 we rejoice. 

But we are called to speak

 for those vulnerable to tragedy.

His daughter was born 4 months after Doug's death

Doug's children in 2010

Their children should not grow up without them.

And their family members should not live in the shadow of fear

 that some day this could happen to them also.

We are
continuing to tell their stories,

raising awareness of the danger,

while living

in a climate of hope.

- the Bicuspid Aortic Foundation

There Are No Perfect Numbers - Making Decisions

In mathematics, there are perfect numbers.

The first one happens to be 6.

 But in aortic disease, there are no perfect numbers.

Just 2 millimeters more

How big is 2 millimeters? It is the thickness of a US dollar coin. Looking at it, one wonders how such a small distance could make so much difference. For one man, it was the difference between having surgery or not. His aorta was 5.8 cm. The doctors told him surgery is done at 6.0 cm. The difference - just 2 mm - the thickness of a coin.

When "6" was the Number
For Louie Lopez, this tiny length became a distance too far, a point never reached. He was in the hospital, talking with the surgeon, when his aorta tore and fully ruptured. Massive bleeding, massive damage. Rushed to surgery, he had no chance. The year was 1995. Yes, "6" was "the number" for aortic aneurysm surgery back then.

Not long ago, Terry Lopez talked about that time, almost 20 years ago, when she lost her husband, Louie. "They were waiting for just 2 mm more. Just 2 mm....surely, 20 years later they can do better than that."

Reading papers, watching medical conference videos from thought leaders in thoracic aortic disease, I marvel at the paucity of answers, the preponderance of uncertainty still, as they continue to debate aneurysm size and the timing of surgery. There is still more unknown than known. Aortic disease remains a more than worthy opponent for those who do battle with it.

The Numbers

Physicians look for numbers to guide them in making decisions about aortic disease. For aneurysms, the most prominent number is the size (diameter) of the aneurysm. There is a great deal of debate about the number, the size when surgery should be done. Some argue to protect from what can be serious complications of surgery, urging that patients wait for the aorta to grow larger. Others promote earlier surgery, at smaller sizes, to protect from life-threatening, deadly events.

The following paper from 2013 is an example of the debate, giving pro and con perspectives from physicians around the globe, Germany, the US, and Australia:

"Aortic Surgery for Ascending Aortic Aneurysms Under 5.0 cm in Diameter in the Presence of Bicuspid Aortic Valve",  authored by Klaus Kallenbach, MD, PhD,, Thoralf M. Sundt, MD, and Thomas H. Marwick, MD, PhD, MPH. 

In this paper, it is noted that very few patients were studied (with differing underlying causes of aortic disease), in coming up with the number for surgery, although used to guide the care of millions.  "The number" has generally been coming down over the years as surgery became safer, but the uncertainty of what the number should be remains.

Some individuals suffer aortic tearing, perhaps death, at smaller sizes. On the other hand, surgery has risk, and offering surgery at smaller sizes may injure someone whose aorta would not have torn or ruptured if allowed to grow larger.

 The size of the aneurysm alone is just not enough. Other measures, other ways to understand, are needed, but not available today.

If there were "perfect numbers" for the aorta, our friend Richard would not have dissected at about the same age and the same aneurysm size as my husband (who did not dissect, but had preventive surgery at 5.2 cm). Richard barely survived and has paid a high price in additional surgery because of his original dissection 10 years ago.

For the narrowed aortic valve, aortic stenosis, there are also numbers: the size of the valve opening and the pressure build up. There are measures for the leaking aortic valve also. Are they perfect numbers, that each individual can trust with their life? Is there a promise that nothing bad will happen before you reach the "guideline" numbers? Nothing bad will happen until you have obvious symptoms?

If there were "perfect numbers" for aortic valves, Chuck Doherty would still be with his family. So would  Chad Rogers.

Guidelines, Not Guarantees 

Those with BAV and other forms of aortic disease need to understand there are no guarantees, no solid promises. There are guidelines based on information available today. To our surprise we may find them quite limited, once we understand what they are based upon. As a patient, you need to research for yourself, understanding as much as you can.

Making Decisions
Part of that research can be searching for and choosing physicians/surgeons with a philosophy and approach to risk that is in agreement with your own. There are things to think about, such as your lifestyle, frequency of foreign travel, and general comfort with the risk of surgery versus the risk of waiting. These decisions should be individualized within the context of guidelines and statistics; decisions made in conjunction with physicians whose philosophy and approach to life and risk are in harmony with your own. Physicians who clearly share their outcome statistics with you.

Handling Uncertainty, Owning Decisions
In 2013, Aortic Valve and Ascending Aorta Guidelines for Management and Quality Measures were published. Including references, there are 66 pages. In the summary on page 54 are these words,"The choice of the best procedure or valve is dependent on many factors as discussed above and no procedure or device is ideal. Ultimately it is up to the patient, the cardiologist, and surgeon to reach a decision on appropriate treatment." 

As these guidelines tell us, it is very important that you, the patient, are a partner in these decisions, and that you understand the basis on which you and your physicians together are making these decisions.

Wouldn't it be wonderful if there were perfect numbers, perfect devices, and simple decision making? Yes, but as in much in life, that is rarely the case. There are pros and cons, trade offs, in most decisions that must be made. But we only have one heart, one aorta, so these are very important decisions.

The more you know, the more you understand the pros and cons, you can make decisions that belong to you, that you can own. Not knowing leaves us vulnerable, caught by surprise. Perhaps many of us have already been there, rudely discovering the imperfections and limitations regarding aortic valve options.

Over 20 years ago, the mechanical valve that saved my husband's life was called a lifetime solution. The night I walked into our home and found my fallen husband, terribly injured by that valve and its complications, I began to understand the terrible depths of untruth in that "promise" that we had happily once believed. I will never forget him saying to me, "I did everything I was supposed to do."

If you understand there are no "perfect numbers", no "ideal" devices and solutions, it will at least help you to be proactive, to question, to research, to seek more opinions when things don't make sense, and to partner with your physicians in the decisions that are made.

And when something does not feel right, when an echo or other test results do not make sense, don't let it go. Keep asking questions, seeking answers.

No one cares more than you do.

 Because it is you, the patient,

 above all others,

 who will "own" the outcome.

May this help you to read,

 question,

 and make informed decisions,

while living in a climate of hope.

Best wishes to all,

Arlys Velebir

Bicuspid Aortic Foundation

Ellie - A Life at Risk

Ellie (not her real name), this is for you, 

and every other person in the world

 with a heart murmur, chest pain, and aortic aneurysm,

 who is not receiving the attention and care they need. 

Ellie lives in a small city in the eastern United States. Ellie has health insurance. But Ellie is not receiving the care she needs. Her life depends on her own strength and determination, her willingness to push through ignorance and find the expertise she needs.

Ellie sought help because of chest pain. Her blood pressure was also high. She has known about a heart murmur for about 15 years. She also knows that her father died suddenly of a "burst heart". He was younger than she is now.

Heart murmur, chest pain, high blood pressure, early sudden death in the family - any alarm bells going off yet?

Ellie had both an echocardiogram and a CT scan. But she did not hear back from the cardiologist who ordered them for 6 weeks. Her calls to him were not returned. In desperation, she asked for an appointment with another doctor, this time the heart surgeon at her local medical center.

From the meeting with the surgeon, Ellie came away with at best confusing and at worst, potentially deadly information.

Ellie was told that she was not a candidate for surgery yet because her aortic aneurysm was not big enough. Ellie is a petite person, but even more importantly, she has chest pain and "heart-related" sudden death in her family.

The following paper was published in 2005.

Symptoms plus family history trump size in thoracic aortic aneurysm.

Why wasn't Ellie told about this?

Perhaps it was when she pressed him, hoping to prevent a catastrophe like her father's, that she was told she "would not come off the table alive" if she had surgery now. Really? What would make the surgery safer later, if Ellie is still alive when it reaches a larger size? At that point, Ellie felt she was given a death sentence.

She was also told that if her blood pressure would come down, her aneurysm could completely go away. Assuming she has BAV, there is nothing in the medical literature that supports this. Where did the surgeon get this misinformation? There is a recent article about a class of blood pressure medication (ACE Inhibitors) given to those with BAV:

The Effect of Angiotensin-converting Enzyme Inhibitors on the Rate of Ascending Aorta Dilation in Patients with Bicuspid Aortal Valve.  

The article concludes "In an adolescent and young adult population with an isolated BAV, there is no proven benefit to ACE inhibitor use with regard to slowing rate of ascending aortic dilation."  There is a need to keep looking for medical answers, but it is important to know that at this time, nothing has been found to prevent or make an aneurysm go away in those with BAV. If that day comes, it will be shouted from the rooftops, and just in the US alone, sales to the millions with BAV will skyrocket.

Does Ellie have BAV? Well, no doctor has explained that to her clearly either. Everything about her suggests it. Why didn't someone explain what is going on with her aortic valve to her?

Ellie's local center does not do complex aortic surgery. Why wasn't she referred to a major center that does do it? Instead, Ellie was advised to go the local ER if she has chest pain again. She was given papers that describe how life-threatening aortic aneurysm in the chest can be. One wonders if that is the handiwork of lawyers, for the protection of the physicians and the hospital. Documentation that the patient was warned of the seriousness of this. It is difficult to see how words on paper can protect Ellie. If Ellie's aorta tears, could she make it to the local ER, have a CT to find the problem, survive a life flight to another center, and make it through emergency surgery? 

What Ellie needs is real help before something deadly happens. So far, she is not getting it. May she find the strength within herself to go to a major center on her own. She needs to find someone who understands her, cares, and has the skill to save her. Until then, may that weak, bulging tissue hang on. If Ellie's aneurysm should tear or burst, with every heart beat she will bleed uncontrollably inside.

Ellie, there are answers and treatment for you.

Please don't give up until you have them.

Ellie happened to cross paths and share her story with someone who volunteers with the Bicuspid Aortic Foundation. Otherwise, we would not know about her. It is an important reminder, lest we forget, that those like Ellie are vulnerable everywhere, despite high tech advances in the treatment of those with aortic disease.

Escaping Dr. Hodad

Dr. Marty Makary, in his book Unaccountable, writes about Dr. Hodad in the very first chapter. Reading about this doctor, I realized that I had heard of him more than once, in more than one hospital. I just did not know his last name, given to him by surgical residents - HODAD - Hands of Death and Destruction. His name says it all. If you are introduced to him, sign no papers. Escape!Run!

Escaping Death

"I thought you would be dead now."

How could a physician, trusted to protect life and do no harm, look directly at her and say this to Rose? But he did. Who was he? He was not a surgeon. He was a medical doctor. But he was part of the team in a terrible surgical drama that played out in Rose's chest, and he did nothing, nothing at all, to help her.

Yes, Rose fell into the hands of Dr. Hodad and those who collaborated with him. But she did not die. From her story, we can learn why it is worth doing everything possible to avoid the hands of death and destruction in the first place.

Every day, somewhere in the world, someone is having a surgery like Rose needed. There are surgeons who have spent many years perfecting the most complex surgery in the chest, aortic surgery, along with replacing the aortic valve. Rose had been born with BAV, and she also had an ascending aortic aneurysm.

Referred from doctor to doctor in her local city, Rose trusted the doctors she met, trusted them with her life. She believed that she would be referred to the right surgeon, with the right skill. She entered the hospital and had surgery. It was far from ideal, but she came through the complications. With time, she should have been feeling better and better.

Something is Terribly Wrong

But Rose was getting worse. She did not need anyone to tell her that something must be terribly wrong. And it was. Eventually Rose learned that her aortic aneurysm surgery had been botched. Normally a Dacron graft is used to replace the bulging section of aorta that is called an aneurysm. But not in the hands of Dr. Hodad. He had replaced her BAV with a pig's valve, which included part of the pig's aorta above the valve. Dr. Hodad simply cut out Rose's aneurysm, and instead of using Dacron, pulled Rose's own remaining aorta down until it met the pig's above the valve. What went wrong? There was too much tension on her aortic tissue. Rose's stretched aortic tissue began to pull away from the stitches holding it to the pig valve, and blood began to leak out around her valve with every beat of her heart. 

I am amazed at the depths of courage and strength Rose found within her. She fought for her life by researching and reaching out to others, asking questions. She trusted people she did not know. They helped her find her way to an experienced aortic surgeon in another state. She put her life in his experienced hands. Rose's first surgery, in those experienced hands, would have been easy.  This surgery was a fight for life. It took all day. He saved her. 

Escaping Destruction
I remember being told, I think by the anesthesiologist, that the surgeon who would replace my husband's critically narrowed BAV was a good technician. That was over 20 years ago. I didn't appreciate it then as much as I do now. Knowing now how complex that surgery was, I know that the surgeon was at least good enough to get through the challenges - a man in heart failure with a critically narrowed, extremely calcified, and abnormally small BAV. I can imagine a very different outcome with Dr. Hodad. Although, as Dr. Makary writes, the best technician may not be charming like Dr. Hodad, it is important to get the very best skill available to you to save your life.

Years later, my husband had aneurysm surgery. I remember a nurse telling me that his surgeon had "golden hands" and would be her choice. I didn't fully understand the importance of what was said. This was not just being polite. This was someone who worked there and saw the outcomes, day after day. Dr. Makary writes about asking the "insiders" about the doctor you are considering. Those who work there will have more information than you do, if you can find a way to tap into their world.

Finding "Dr. Right"
Often the Bicuspid Aortic Foundation is asked about physicians who can help those with BAV. This is such a difficult dilemma for us all. We do not have enough information to help find Dr. Right in the many places where he is so needed. What we can do is encourage everyone to do everything they can to find Dr. Right and flee from Dr. Hodad.

For those with BAV, medication cannot solve their problems, and surgery and surgeons are extremely important. Thinking about Rose (not her real name), my own family, and the larger genetically related BAV family whose lives all depend on surgical skill, there are things we can do to help ourselves:

• Referrals given may be to Dr. Hodad, not to Dr. Right; you can refer yourself to surgeons in order to find Dr. Right
• Educate yourself about the procedure you need and ask the surgeons you consider many questions 
• Think in terms of the best skill based on results, not the nicest personality 
• Other patients may not know enough about what happened to them; ask the "insiders"

Together,
We are
Creating a 

Climate of Hope

Arlys Velebir

Bicuspid Aortic Foundation

Lily's Story - Bicuspid Aortic Valve Stenosis

Broken Heart Lily
 (picture from David's Garden Diary Blog)

I have never met Lily in person.

I have never seen her picture. 

I know the sound of her voice.

And I know her heart.

I will never forget her.

Meeting Lily
Lily contacted the Bicuspid Aortic Foundation one day. She told me that she had severe stenosis (narrowing) of her bicuspid aortic valve (BAV), as well as an aortic aneurysm starting. The narrowing was because over time Lily's bicuspid aortic valve leaflets had become calcified and stiff. They do not open widely any more, and are getting worse.

Yes, Lily was born with a bicuspid aortic valve. But that was not all. Like others with BAV, she entered the world with "delicate tissue". Numerous health issues have surfaced over her lifetime, at least some genetically based. Yes, whatever is underlying it all, Lily has lived with the pronounced effects through out her body. And yet, Lily married and had a family. She has clearly been an active woman with many interests in her life.

Lily sent me a list of her medical conditions. There are over 30!

Yet, of all her many medical conditions, it is this tight, narrow aortic valve that now threatens her life.

Lily's doctors spoke to her of options, but there are not many. She is very high risk for surgery. Lily wanted very much to know about someone else like her, someone who could not have surgery. This is the longing to be understood, to not be so terribly alone, to know someone cares.

Could TAVI Help Lily? (transcatheter aortic valve insertion)
What about the new option of inserting a tissue valve inside an existing calcified aortic valve without opening the chest? It is meant for those, like Lily, who are too high risk for surgery. But in the United States it is not approved for those, also like Lily, who have a bicuspid aortic valve.

Recently, Lily wrote that she was going to investigate a clinical trial for TAVI in those with BAV. She was seen quickly, as her symptoms are getting worse.

Just two days ago, Lily wrote again, explaining that she does not qualify for this either because of the shape of her particular bicuspid aortic valve,

 ". . . I needed to have a tricuspid valve that two flaps had fused together instead of a true bicuspid that is missing one of the three flaps altogether making it a mouth shape instead of a 3 points shape. It needs the extra point to hold it in."  

In BAV, A Disease of the Elderly in the Young 
Aortic stenosis is a terrible scourge on society. One doctor wrote an editorial calling severe aortic stenosis a "neglected malignant disorder". It often is thought of as a condition of the elderly. However, BAV hearts are prone to it at younger ages. BAV is the most common reason for aortic stenosis in those under the age of 65. Usually, the narrowing of the valve is caused by gradual calcification. However, there are some infants and children with BAVs narrowed abnormally by tissue, right from birth.

In the United States alone, statistics showed there were 300,000 individuals in need of surgery for aortic stenosis, but in one year only 80,000 received it. (There is no medicine to cure this.) Without some kind of intervention, the rest will die. Once it reaches a critical point, half are dead within two years. (View the videos in this post from 2011 for more information.)

Broken Heart Lily
A double oriental lily
whose fragrance
 is exceptionally sweet

Lily's Courage
Lily's symptoms are getting worse. She is no longer allowed to drive. She ended her note like this,
"I feel I have exhausted all my options and at peace that I have done all I can do. I am enjoying every day with blessed hope and contentment.

Love n Prayers, Lily"

Lily is one of those thousands in the United States
for whom there is no answer. I have called her Lily, which is not her real name. She is a courageous, strong woman, too young and too beloved to be taken from her family.

In honor of Lily's courage and strength, let us continue to raise awareness and to press for ways to mend beautiful, broken hearts like hers.

Love to you and your family, dear Lily,
Arlys Velebir
Bicuspid Aortic Foundation

Bicuspid Aortic Valve and Aortic Disease Answers Are Blowing in the Wind

Kyle Wilson
sudden death
BAV and aortic dissection

Chuck Doherty
sudden death
BAV & aortic stenosis

"Yes, how many deaths will it take 'til he knows

That too many people have died?

The answer my friend is blowin' in the wind,

The answer is

blowin' in the 

wind." 

                                  - Bob Dylan, 1962

Doug Grieshop
sudden death
BAV and aortic rupture

Friday, we received that most dreaded message once again.

Because we know those with BAV very well,

reading the words,

we feel we know this person, born with BAV.

". . .  very athletic."

". . .  so healthy."

We hold our breath . . . .

"He is dead."

And then we cry.

How many, oh, how many, must die?

Sometimes we read of their tragedy, although we may never meet these families. 

Football player who died in Knoxville had problem heart valve, WATE.com, November 19, 2009

Coroner releases what caused Chad Rogers' death, Fox4KC.com, September 4, 2013

Orion boy, 4, loses quest for new heart, QCOnline.com, July 30, 2012 

Sometimes, a bicuspid aortic valve has been found,
 and the person was under medical care.

Sometimes the BAV had not been diagnosed.

In both scenarios, there is death.

Why, oh why?

Sahib Singh Vaseer
death from
BAV and endocarditis

We are left to ponder very serious questions.

Where are the answers?

They are as ethereal 

as wispy white clouds,

driven by the wind.

Other families, like mine, avoid death
 but cope with life-long injuries,

both physical and emotional.

That is why Robby Benson, 

a veteran of four open heart surgeries,

entitled his book

"I'm Not Dead Yet ... !"

A recent paper from a consortium of centers studying BAV, with authors from around the world, is aptly titled, "Surgical treatment of bicuspid aortic valve disease: Knowledge gaps and research perspectives."

We simply cannot tolerate, cannot bear the tremendous ache with every injury, every death in BAV families. Yes, their trileaflet aortic valve family members also are at risk. These knowledge gaps are not small things.

Those pictured here speak to us of major complications that may strike down even the young and strong - the aortic valve itself, aortic dissection, aortic rupture, and infection (endocarditis).

To remind us all of the tremendous problem, and our shared responsibility to conquer this centuries-old monster, this blog has adopted "Blowin' in the Wind" as our theme song. 

Glimpses of small 
numbers of people, 

watched only

for a few short years,

Fragments of information

blowing in the wind,

Will not answer our questions,

will not satisfy our needs.

May we all resolve

to take action,

to free humanity at last

from this plague.

                                                                                                 - Arlys Velebir

                                                                                                   Bicuspid Aortic Foundation

Emerson - Our Miracle Boy

Baby Emerson was diagnosed with BAV and AS at 11 weeks

Statistics say that a baby with BAV enters the world every 12 seconds. We also know that they are more likely to be a little boy rather than a girl. Some of them may live many years before their BAV is even discovered. Today, there is increased focus on these adults with BAV and their families.

But there is a group of babies born with BAV who need attention and intervention while still infants or children. They are very much in need of understanding, care, and advancements to help them also.

Who are these little ones, and what is done to help them? 

Statistics may help put the frequency in context, but numbers can be so cold and impersonal. They can never convey what it means to these little ones and their families to face seemingly endless doctor visits and surgery. In order to express the true meaning of BAV in infants and children, we must share their stories.

And so, with the help of his parents, here we share the story of a beautiful little baby, born just a few months ago - Emerson - Our Miracle Boy

Thank you, Nicole and Kyle, for sharing him with us.

 May we all learn the lessons

 that Emerson has to teach us

 about little ones affected by BAV,

Creating a Climate of Hope.

Journey of the Heart

Dr. Sharo Raissi, Lia Nicandrou, Arlys Velebir, Father Prodromos

At the Bicuspid Aortic Foundation, we are very touched and encouraged whenever we hear from those dealing with BAV and the complications of thoracic aortic disease. We take our mission very seriously, knowing that all around the world we share a common experience with families who have BAV and variations of thoracic aortic disease.

The story we share here, of one young man's journey seeking answers and help, spans the many miles between the island of Cyprus and southern California. It would seem highly unlikely that a young man living in a monastery high in the mountains of Cyprus would find his way to the Bicuspid Aortic Foundation. It would seem even less likely that doors would open that would make it possible for his surgery to be performed here. And critically important for this young man, he would find himself in hands that not only could deal with his ascending aortic aneurysm, but also with the strands of tissue clinging to his 13 year old mechanical valve.

His story, in his own words, is on the BAF website - Journey of the Heart .

We are very grateful that Father Prodromos has so graciously shared his experience with others,

Helping us in our mission, 

and creating a climate of hope,

Arlys Velebir

Chairman and President,

Bicuspid Aortic Foundation

Alicia's Story - From Fatigued and Fainting to a New Life!

Alicia, 3 months after surgery, hiked 3.1 miles!

Her Mom remembers Alicia as a healthy baby. At the age of 7 she developed asthma and was also hospitalized with pneumonia, suffering staff infections. From then on, all was well as she grew and went through her teen age years.

But things changed for her at the age of 20, following the birth of her first child, when she began to have complications.

It was during a routine exam that her doctor heard a heart murmur and suggested she see a cardiologist. At her first cardio exam, she was diagnosed with a bicuspid aortic valve. She was told she was fine and would need to be monitored annually. But she had some difficulties during her pregnancy - dizzy spells and fainting. She gave birth to her daughter in 2009.

Her baby daughter was very ill during her first year of life and was ultimately diagnosed with a PDA (patent ductus arteriosis). At one year of age, the PDA was closed, resulting in a very healthy child ever since.

But for Alicia, all was not well. She tired easily, was short of breath, and often felt faint. When she visited her cardiologist in May of 2011, she was told that nothing had changed.

She was also told that she likely would not need heart surgery until she was in her 40's.

How could she face the next 20 years feeling like this? 

The new year, 2012, found this young mother lightheaded, tiring easily, sometimes fainting, and suffering from migraine head aches. Her cardiologist told her she needed to live with these symptoms, and that she was too young to have surgery. She was also cautioned not to have more children.

The months passed, and the symptoms persisted. Alicia was extremely fatigued and fainted often, also suffering frequent migraines. Her cardiologist prescribed metroprolol for her migraines, which lowered her blood pressure and made her feel worse than ever. Alicia had chronically low blood pressure, and the metroprolol took away what little energy she had.

Their search for help led this family to the Bicuspid Aortic Foundation, looking for information, support, and hope. Alicia sought a second opinion from Dr. Sharo Raissi, who chairs the Foundation's Scientific Advisory Board. She underwent testing that revealed she had an aortic aneurysm along with her BAV. It was clear that Alicia continued to feel unwell, and she could not keep up with her very active toddler. In the fall of 2012, after having further tests, plans were put in place for surgery to remove the aortic aneurysm. The bicuspid aortic valve would be replaced if necessary. Amazingly, in December Alicia completed an extensive pharmacy technician program, despite feeling very tired and weak.

Alicia had surgery on January 16, 2013, at St. John's Health Center. The aneurysm was removed and her bicuspid aortic valve replaced with a bovine prosthetic valve. She now looked forward to feeling better, as her body healed.

By March, she was doing very well indeed. Alicia proved her new found energy and stamina by completing a 3.1 mile hike just three months after surgery (pictured above). What a dream come true! Today she feels great and is so happy. She now knows what it feels like to be the healthy woman she was meant to be! And her daughter notices it too. For the first time in her three years of life, she has a healthy, energetic Mom. This is a very happy family!

Alicia is now well informed about her health. She knows that some day the bovine prosthetic valve will require intervention. In the mean time, she is doing all she can to take charge of her health and her life!

May Alicia's story inspire others to listen to their bodies, and seek the help they need until they find it.

Thank you, Alicia, for sharing your story, and creating a climate of hope.

Paying It Forward - Touching Many Hearts

From One Heart

To Another

"When you helped me, you helped many people......"

Pay It Forward!

Invisible Bonds of the Heart
How do you describe those that have walked together through life and death decisions? I doubt that there is a word in any language. They are connected in a very special way, through an invisible thread between them. Each end of this thread is tied with a very special knot, deep inside their beings. It is a bond that cannot be broken. Over time, as one person helps someone, and they in turn help others, hearts bond together in an unbreakable web. Each one of us can be a part of this wonderful connection. All by paying forward what someone else has done for us.

I have a connection like this to one particular woman in the mid western United States. Her bicuspid aortic valve and aneurysm surgery failed, and only a few months after her surgery she was in serious trouble. It is beyond my comprehension why her physicians did not help her get to a major heart center. Perhaps they thought she was beyond hope. Apparently one of her doctors thought that, because he later told her he expected she would have been dead rather than coming to see him in his office again.

She was not beyond hope! I helped her go to a well known aortic surgeon in another state, and she is not only alive today, she pays the gift of life forward at every opportunity!

Recently, I visited a young man in the hospital. He had just had a much needed surgery. The valve and aneurysm that both threatened him can no longer harm him! He found his way to help not through referrals from physicians, but through his own diligent research and because this special woman reached out to him and pointed the way to help.

This young man is one of several that she has helped. In a recent email, she told me about them. She closed her email this way,

"These are all people who have a chance to live long lives, 

because they are getting expert care.

That's what you taught me, Arlys, that there's care,

 and then there's expert care.

I wouldn't be alive right now, if I hadn't had expert care, so thank you."

You too can pay it forward.

After first getting expert help yourself, share it with another. 

In this way, all around the world

 we join together in this

 indescribable connection of hearts,

Creating a Climate of Hope.

Best wishes,
Arlys Velebir
Bicuspid Aortic Foundation

Chuck and Cheri Sheridan - Aortic Dissection and Hope

 by Marie LoParcoCheri wrote that this artist's proof, created by her friend, is to be shared with others as an expression of hope. It is fitting that a picture of it be shared here, with their story.

 This story of hope was written in  May 2007 by Cheri Sheridan about what she and her husband, Chuck, experienced almost three years earlier. 

Before you read Cheri's words, I will add a little background to this story. At the time Cheri writes about in 2004, I was privileged to work with Dr. Raissi on a limited basis in his Aortic Surgery Program. And that is how I know Cheri and Chuck.

I still have not met them in person, but I know Cheri especially well. We have walked in the same shoes. The first time I heard Cheri's voice, I recognized in it the timbre of fear that has at times gripped me also.

 Aortic disease does that. An icy fear penetrates to our very core. 

It is the fear of losing our loved one in a fight with an enemy we scarcely understand. Cheri mentions being "frozen with fear". 

She was afraid there was no hope. 

This is one of the reasons that the Bicuspid Aortic Foundation emphasizes creating a climate of hope. Hope empowers us to keep seeking  help. Cheri and Chuck did find hope. They found choices they never knew existed and skilled hands to save Chuck's life.  

Today, as you are about to read, Chuck and Cheri "have their life back". Thank you, Cheri and Chuck, for sharing your lives here and giving hope to others. We all need hope so much. - Arlys Velebir

The rich, warm colors that the artist chose for this work,
seen in this close up, remind us that icy fear melts away in the warmth of hope.

                                                                           
                                                                         May 2007
                                                         Here is Chuck and Cheri's story . . . .

I am writing this almost 3 years after the fact: I would like to express my feelings about the compassion, encouragement, and care that we personally received from Arlys and Dr.Raissi at the Cedars Sinai Hospital. My husband had had 2 surgeries after having an aortic dissection while swimming in 1996. It was a miracle that he survived his first episode. Several years later after another operation repairing another segment of his aorta his yearly CAT scan showed that he was in need of yet a third operation. His medical reports were sent to Cleveland Clinic without our knowledge and a physician's secretary left a message on our answering machine saying that my husband needed to get in touch with that doctor immediately for a consultation.

We did and that doctor told us that my husband needed to have 2 sections of his aorta that were previously repaired redone in two more radically invasive operations, to be scheduled a month or two apart. When my husband asked what his options were, he was asked by the doctor if the word mortality meant anything to him. It was a very long drive home to central NY from Cleveland, Ohio. I was frozen with fear, believing that my husband would not be able to "get his head around" two more invasive surgeries and that I would loose him.

Fortunately for us, when we were at the Cleveland Clinic that day, waiting to see the doctor, we met a nurse who had had the nearly same experience that my husband had had in the same year. We spent the entire day together waiting to see the doctor. It was through her, after comparing similar experiences that we finally learned what much of the medical jargon meant. She gave specific details about stroke, heparin causing bleeding ulcers, and how low her blood pressure was being kept and what specific medications/doses that she was taking. I wrote everything down as I had been doing with each person that my husband had met within the 2 days of testing/evaluating procedures prior to our meeting with the doctor.

Still not knowing what to do, several days later I found the Cedars Sinai Web page and discovered it to be very easy to navigate. It even had a space to write in a specific aortic question. It also offered a page that you could go to that would answer general questions that were asked about aortic medical problems and I knew that each aortic dissection survivor has different/individual circumstances. So I wrote in something about our situation; it was processed and shortly I received an email that said a Cedars Sinai Hospital liaison person would be in touch with me within 24 hours.

That happened! Arlys called me and she asked me how she could be of help. I told her our situation and I said to her that if we were "just tilting at windmills and didn't have any hope" that someone should tell us and that we would just live out our lives in the time that we had. She said,"Oh no! You are only looking for the least invasive procedure that you can find for your husband…you have so much hope and many possibilities!" She gave me her work number and her cell number and remained in constant contact with us and introduced us to Dr. Raissi. 

We sent all of the medical reports that we had received about my husband's condition to him. He spoke personally to my husband, our local family doctor, and me. Over a period of 3 months he got my husband's blood pressure to a safer level and referred us to medical help on the East coast near our children who both lived in NYC. Arlys and Dr. Raissi never let go of us! We also knew that if we didn't find the medical care that we were looking for in NYC that Dr. Raissi would do the operation. We felt their care and concern immediately from the first time I heard Arlys' voice.

We had the surgery in NYC and it proved to be successful. Arlys and Dr. Raissi directed and guided us through a very rough time in our lives and held us close. My husband is back in the pool for exercise, just won his division in our local Canoe/Kayak Race, and works full time. We will forever be grateful that we have our lives back!  - Chuck and Cheri Sheridan