A Happy 7th Aneurysm Surgery Anniversary

Looking back,

It almost seems like a birthday,

 that day we had aortic surgery.

Perhaps it was for our valve,

an aneurysm in our chest,

or both.

Some of us have several of these days.

It is a day we always remember!

There was a very special message for the Bicuspid Aortic Foundation recently. I share part of it below:

"...2008.... New York Presbyterian Hospital....at this hour, I was under the knife for my third (1st being in 1967, 2nd being in 1975) open heart surgery...this time to replace my ascending aorta and aortic valve due to a large aneurysm...and I guess the surgery was successful since I'm still here and kicking!

Thank you to the Bicuspid Aortic Foundation (www.bicuspidfoundation.com) for without your website, I would never have suspected that I had an aneurysm."

This man had been under cardiac care for many years, since he was quite young, but no one had ever told him that someone born with BAV was at risk for developing an aneurysm. After reading information on the Foundation website, he asked his cardiologist about it. A CAT scan was done, and there was the aneurysm! He feels that the aneurysm was so large, he would not have survived to see the new year of 2009!

How much we all would have lost if this very active, vibrant man's life had been needlessly cut short.

Please, tell everyone you meet about BAV and aneurysms. BAV is very common! You can save lives!

In telling others to get checked,

We may unmask a silent threat,

Creating a climate of hope. 

Best wishes to all,

Arlys Velebir

President & Chairman

Bicuspid Aortic Foundation

Doctors and Hospitals with Heart Problems

Where can I find the best physicians, the best surgeon, the best hospital?

 These questions confront us all,

 who have BAV and TAD in our families. 

It is a question we must deal with

 from the first day it is found,

 for as long as we live. 

It is extremely important to be cautious about where our trust is placed.

 We ache for these families and the injury and loss they have suffered.

Coarctation of the Aorta

Little Layla McCarthy, who lives today with paralysis, had surgery to "widen her aorta". Strictly speaking, this is not heart surgery - it is surgery on the aorta, just past the arch. Those with coarctation often also have a bicuspid aortic valve (BAV). Repairs to this section of narrowed aorta may cause paralysis, if blood flow to the spinal cord is impaired. Parents should be told about this risk.

Here is the link to a full paper that discusses informed consent for heart surgery in children:  Procedure-Based Complications to Guide Informed Consent: Analysis of Society of Thoracic Surgeons-Congenital Heart Surgery Database  Notice this sentence about coarctation: "While complication rate thresholds may guide the clinician, rare but important debilitating complications, such as paraplegia after coarctation repair, should also be discussed irrespective of frequency." Yes, it is important to know everything. 

Searching for Answers

This is why the Bicuspid Aortic Foundation puts great emphasis on researching physicians and their associated hospitals. When individuals and families meet with physicians, we need to press them for answers to the hard questions, do our best to find out what could possibly go wrong. In addition, we should do everything possible to independently confirm the answers we are given, 

It is not easy to discuss these things, but how much worse to have a false sense of security and complacency. It is not helpful to hear phrases like "it's a walk in the park". We need solid information about numbers of the exact surgery performed by the surgeon we are considering, and the complication and death rates involved. We also need to know about infection rates for the hospital.

No matter what our age, from infancy to later in life, we must seek the very best to care for us. Some day, perhaps there will be more openness, more transparency. Until then, may we all do what we can, and make every effort to ask probing questions and seek answers until we are satisfied we have done what we could. 

Walking this BAV/TAD road together,

sharing with one another,

we create a climate of hope.

Best wishes,

       Arlys Velebir

                          Bicuspid Aortic Founation

Sharing Courage

A few times, I have wondered who was more afraid, my husband and I or the people, the medical professionals, we met along the way in his journey with BAV.

I wrote the verses below after my husband's aneurysm surgery. Recently I dusted them off.

He was in the hospital on a heparin drip before that surgery, because he had a prosthetic mechanical valve and had to be weaned from warfarin. I remember very well one doctor who visited his room the night before surgery. We were feeling at peace, looking forward to soon being aneurysm free! We both remember one part of the conversation with that doctor very well. He crossed his fingers on both hands and said "of course, anything can happen." Well, we did not catch his fear, but we were in a tough spot, pretty well trapped by signed legal papers and that heparin drip, if we had panicked. He was a medical doctor, not a surgeon. Today, I remember this doctor with compassion and have always hoped he will never need aneurysm surgery himself. If he does, maybe he will remember the many excellent results from surgeries like my husband's and take courage.

A nurse said the words "time bomb" to me during my husband's surgery that day. Somehow meant to console me, an acknowledgement that there was no choice, those words were jarring to my own heart. We were not in denial. We knew why he needed the surgery he was having that day. We had waited, since we found out it was there, for this day when it would be removed. 

Since then, I have spoken with many people about their aneurysms and upcoming surgeries. I have had people ask me, some whom I have never met in person, if they are going to make it.

I have never once spoken about time bombs. I have never crossed my fingers in front of those I meet. I have met them with a smile, not because I do not know how serious it is, but because we are in this fight to win. No one should face surgery already feeling defeated. 

I understand now, years later, that aneurysm of the aorta in the chest can strike medical professionals with fear also. Even when it is not an emergency. And very much more so, when it is.

The lines below are a call for courage in the face of this tough opponent, aortic disease in the chest. I have learned that not just aneurysms, but valves, and infection, may be challenging too. My husband has had three surgeries, and the two involving his valve, for different reasons each time, were difficult, risky.

It has been said that courage is not the absence of fear. It is taking action in spite of fear.  Please, share your courage, not your fear.

This little poem is meant to speak for the person having the surgery. It is meant for everyone who knows or will meet someone facing that surgery, for an aneurysm or some other complications of aortic disease in their chest.

  Sharing Courage

Please don’t tell me I have a time bomb in my chest

Don’t let me see fear in your eyes.

I cannot forget, yes, I know it is there,

This terror within, in disguise.

Please tell me the best is all there for me,

Share your strength, understanding, and care.

With your help and support I’m reminded

This terror within, I can bear.

Please tell me the wonders of surgery,

Tell of the many it’s saved.

Many days I have lived with this enemy,

This terror within, I have braved.

Please tell me together we’ll do this,

Remind me today is a great day.

No match for the hands of the surgeon,

This terror within cannot stay.

Please tell me you’re there for my family,

Share your courage, assure them it’s true.

Waiting now, soon they’ll see and touch me again,

This terror within hurts them too.

Please tell me that soon it can’t hurt me,

Smile and tell me again that we’ll win.

And when I awake, all new inside,

No more terror, my healing begins.

              - Arlys Velebir

Please give yourself time whenever you need it, time when you can cry, seek comfort, and renew your own courage. Then go out again to share your courage with others.

Courage is always,

an important ingredient

When Creating a Climate of Hope.

Best wishes,

Arlys Velebir

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