September Awareness 2022 - The Aorta is Not the Heart!

 

When the Bicuspid Aortic Foundation first chose an Awareness Month for the Thoracic Aorta, it was important to designate a time widely separated from February, "Heart Month". September is widely separated from February on the calendar.

Quite simply, the reason for this was to educate individuals and families about their aorta in the chest as a separate, unique, and vital entity in its own right.  

Especially in emergency situations, but also in office visit settings, when the focus has been on the heart primarily, there have been far too many "near misses" and tragic loss of lives. 

Too often, including the current era, some articles for the general public confuse the heart with aortic disease in the chest. There are inherent dangers in such confusion. The tests and treatment for thoracic aortic disease are very different from that for typical heart disease, which involves blockage of the arteries of the heart. 

It is encouraging in 2022 that there are global efforts to inform the public about aortic disease in the chest.

Once again, in September 2022,

 we applaud and join with the efforts of all

 who accurately educate and inform

 the public about the aorta in the chest,

Creating a Climate of Hope,

~ The Bicuspid Aortic Foundation 

September Awareness 2021 - Why "TAD" Awareness

2001  and "TAA" (Thoracic Aortic Aneurysm)

My personal involvement with aortic aneurysm in the chest began 20 years ago, in 2001. There was so little public information then. My late husband's physicians, although carefully monitoring his anticoagulation levels, had not  followed his aorta in the years following his BAV replacement. Believing a mechanical valve equaled "fixed for life", we were comfortable with only a very few echocardiograms in the following years. We were spared an aortic catastrophe when one of those echocardiograms, to check on the 11-year-old mechanical aortic valve, "discovered" the bulging aneurysm above it. 

2005 and Thoracic Aortic Aneurysm and Dissection (TAAD) Awareness

We read medical references in our search for information and help in 2001, where we found an unfamiliar term, "aortic dissection".  By 2005, when the Bicuspid Aortic Foundation first observed awareness for the aorta in the chest, we had learned about aortic dissection, the "D" in TAAD.

We learned the medical definitions, yes, but much more, we learned the meaning in human terms, for individuals and families. We do not forget those we met in those beginning days.

It is the human experience that ultimately defines these medical words:

 those who die, 
those who survive with injury,
 those who have elective surgery with desired outcomes.

2021 and Thoracic Aortic Disease (TAD) Awareness

With the passing of the years, at BAF we met others with additional aortic complexities such as coarctation. Again, we had learned the terms first, but their meaning comes from meeting those who have them - from children to adults. Consequently, we have adopted an umbrella term - "Thoracic Aortic Disease" to include them as well. 

One Man's Life-Long Aortic Journey Until Now 

Father Prodromos Nikolau's life experience with TAD began at age 2 in 1981, and thus far has included the following:

• coarctation repair (1981)
• BAV replacement (2000)
• "Thinking Everything is Fixed" (after BAV replacement, annual cardiology visits)
• discovery of Aortic Root Aneurysm (2007)
• root and ascending aortic aneurysm repair and replacement of prosthetic aortic valve due to strands (2013)
• "redo"of original coarctation repair (2015)
• ongoing checkups, life-long aortic care

 At the following two links are the details, in Father Prodromos' own words:

Journey Beginning  from Age 2 

Hidden Danger of Patched Coarctation

"So, life is precious"

 as Father Prodromos wrote,

and life can be extended 

for those with TAD

when they receive

 Life-Long Aortic Care.

~Arlys Velebir, Bicuspid Aortic Foundation

September Awareness - Forever 33 - BAV, Aortic Dissection, Rupture

Doug a few days before his 33rd birthday

Forever 33

Sometimes it seems so cold

 to just write about someone.

 Somehow it feels right to address

 this personally to Doug Grieshop.

Dear Doug,

Tomorrow, September 19th, is your birthday. Can it be 12 years now since you celebrated turning 33 with your family? You should be with them still. Tomorrow you would be 45.  Instead, in your pictures and in loving memories, you are forever that strong, vibrant young man of 33.

We still have the first email Stacey wrote to the Bicuspid Aortic Foundation. The title "My Husband" did not prepare us for those first tragic words "My husband passed away unexpectedly...." It is the message of unspeakable loss that we hope never, ever to receive at the Bicuspid Aortic Foundation. For those who do not know, they can read what happened to you here, Bicuspid Aortic Valve and Aortic Rupture .

Doug's children remember
 Daddy's heavenly birthday

I know that knowledge is powerful, but when I remember you, Doug, I realize just how much power it has. As you took those last steps before you collapsed, you had never heard about bicuspid aortic valves and aneurysms of the aorta. You had never heard of  aortic dissection and rupture in the chest.

It seems so wrong that for months you were fighting something very real that no one understood or named, although it should be well known. Recently Stacey was mentioning what happened once again. She wrote of the multiple specialists you saw. There was a stress test. A chest x-ray, but no CT scan. You were given anti-anxiety medication. Of course, those things did not in any way help unmask the enemy in your chest. It hurts so much to realize that the technology and ability were there. In your own state is a world renowned center for treating this. You just did not know.

Doug, it would have been so wonderful to talk to you on the phone, to meet you in person, as we eventually did with Stacey and your family. We would tell you about your aorta in time, so you could get help.

We cannot change what happened, I know. I just want to tell you that because of you, we have the courage to tell others to persist in getting the help they need, to move on and get other opinions when doctors don't understand.

Doug, you inspire us to save others. 

This is your forever legacy. 

This September, 

we continue

to share knowledge 

Creating a Climate of Hope.

With our love,
Arlys Velebir and all the volunteers
at the Bicuspid Aortic Foundation

September Awareness 2016 - Learning About the Aorta

Richard Houchin (right) running along the route of the LA Marathon
following survival of aortic dissection

"Aorta? What's my Aorta?"

 As he lay on a stretcher, helpless and vulnerable, Richard Houchin remembers thinking this as he heard voices around him in the ER talking about him. Something was terribly wrong with his aorta, whatever that was!  Click here for details of what happened to Richard that day.

This was the way Richard learned not only about his aorta, but that he had been born with a bicuspid aortic valve. It is not the way anyone should learn this.One of the goals of this awareness month is to raise public awareness of the aorta, and how aortic disease can be detected before it threatens someone's life.

Following are a series of pages on the Bicuspid Aortic Foundation to help learn about this great blood vessel, truly a river of life in the body.

1. What is the Aorta?

2. What is a Thoracic Aortic Aneurysm?

3. What is Aortic Dissection?

4. What is Aortic Rupture? 


Along with these pages, here is a video from the Nucleus Medical YouTube Channel which describes the thoracic aorta and aortic dissection.

Learning together, we can be prepared to discuss not just our heart, not just our heart valves, but also our aorta with our physicians.

As we learn,

and are prepared to speak

with our doctors, 

we are

Creating a Climate of Hope.

September Awareness 2016 - An Interview with Aorta and Heart

An Interview with Aorta and Heart

The aorta is the large artery seen rising up from the heart,
 with arteries branching upward to the head, then curving down

Two vital members of our body, Aorta and Heart, have agreed to be interviewed for Thoracic Aortic Disease Awareness Month 2016, hoping to set the record straight. They have so much to say, but for now agree to each limit this interview to their top three concerns. Aorta, being less famous, humbly lets Heart start first.

 Heart's Top Three

1. I am being confused with aortic disease in the chest.  
2. Aorta, you are so important! If you start bleeding around me, I may stop beating.
3. If it's not the Heart, check the Aorta!

Let me tell you and everyone, Aorta, being famous is not such a great thing. Have you done a search online lately, Aorta? The headlines scream about "Heart Problems", when you, Aorta, are the one with the problems. In these articles, aneurysm and dissection are mentioned - this is about you, Aorta, not me. How confusing is that! I know we are both in the chest, but in the year 2016 we should know better. There are enough problems that do belong to me, the Heart, without confusing me with aortic disease in the chest too. This is not just bad for my reputation, it is dangerous for the whole Body. We need to be very clear - is it a Heart attack, or is it the Aorta? Sometimes, in death, only an autopsy can tell for sure. I, the Heart, do not want to be blamed for deaths caused by Aorta!

Healthy Aorta

Aorta's Top Three

1. Nobody knows who I am. 
2. If they do know who I am, they seem to either not respect me or be terrified of me. 
3. Please, fix me before I break. 

People don't draw pictures of me, and I don't have a month with valentines, chocolates, and flowers dedicated to me. I guess no one ever thought that love was in their Aorta! Once you get to know me though, I am pretty amazing in my own right. There is just a lot of misunderstanding about me. Those who know me best do respect me and want to help me.

I have no desire to tear inside, or worse yet, fully break open. I'll admit, I'm not easy to work on, but it's going to be a lot worse for everyone involved if I start to bleed.  I'm so sorry that no one knows just when I may finally reach my breaking point. There are some clues, but knowing when to fix me is still a big challenge today. Maybe if I become a little more famous, more research will finally be funded. 

Thank you for giving me a special month of my own. I don't need the chocolates and flowers, just some attention! Please let me be clear, Heart does have a lot of problems. Just don't forget about me, your Aorta.  I hope September Awareness 2016 will help everyone learn more about me!

End of the Interview

The interview ended with Heart and Aorta acknowledging how close they are, special friends in the chest one might say. In health, they work together beautifully. They each just want their separate identities and problems to be understood, respected, and treated. They both deserve help and attention when they are diseased. After all, no one wants to be known as a killer. 

Before they left, Heart and Aorta enthusiastically repeated together, along with the interviewer, 

If it's not the Heart, check the Aorta! 

Learning Together

We are

Creating a Climate

of Hope!

                                           - Bicuspid Aortic Foundation

September 2016 Awareness Month - It's All About the "Big A" in the Chest!

Normal Aorta

The aorta is so important,

 we call it the "Big A"! 

To baseball fans in the US, the Big A means Angels Stadium in southern California. At the Bicuspid Aortic Foundation, we mean something else; to us, the Big A is the aorta in the chest. It is the largest artery in our bodies, and it's proper functioning is without question vital to our lives.

Why does the aorta need its own awareness month, September?

Maybe the aorta could just share February with the heart?

Tragedies happen when no one thinks about the aorta. In the emergency room (ER), someone with chest pain will be checked for a heart attack. The blood tests come back normal, so is the EKG. It's not the heart.

No one thinks of the aorta.

The minutes go by, turning into hours. Sometimes the person is still in the ER, sometimes they are back home. Suddenly, they are dead. The opportunity to help them was missed. 

All because no one thought about the aorta.

This is one reason that the aorta needs its own month - just to help everyone think about it in time.

Aorta with aneurysm

Another reason is that aortic disease is very different from what is typically called heart disease. The treatment is different too.

Aortic disease is not heart disease!

If it's not the heart, check the aorta! 

Let's Learn All About the Big A this September!

In a nutshell, this large, high pressure artery should not be confused with the heart. Having its own month provides a time to learn about the aorta in the chest: what it does, the risks aortic disease presents to us, and the treatment available when needed. 

At the Bicuspid Aortic Foundation, we will spend this September featuring articles on the following topics:

• Thoracic Aorta - what it is, what it does
• Thoracic Aortic Disease
• Thoracic Aortic Aneurysm
• Thoracic Aortic Dissection
• Thoracic Aortic Coarctation

We will also honor the memory of those who were cruelly taken from us by thoracic aortic disease, and the courage of those who live with injury caused by thoracic aortic disease.

Shining a spotlight on 

the Big A,

Together we are

Learning, Sharing,

Empowering each other,

Creating a Climate of Hope.

Aortic Aneurysm and Dissection Risk from Fluoroquinolone Drugs (Cipro, Levaquin, etc.)

Aortic Aneurysm and Dissection and Fluoroquinolone Drugs 

Raising Awareness - Why?

There have been some additional questions about recent medical papers and the FDA warning about this group of drugs, which the public recognizes by names such as Cipro and Levaquin. Some may have wondered why the Bicuspid Aortic Foundation is raising awareness about this. They may wonder if it has something to do with their heart. It is not the heart, it is the aorta that is getting attention, along with tendons, muscles, and other areas of the body.

To be clear, these warnings are for the general public, for everyone. 

It is helpful to tell everyone about this.

The reason the Bicuspid Aortic Foundation is putting so much emphasis on this is because we advocate for a special group within the public at large, those who already are prone to developing aneurysms of the aorta in their chest.

Quite simply, there are important questions that we must ask:

Ascending
Aortic
Aneurysm

• If it puts the general population at risk of aneurysm and dissection, what about those who already have enlarged aortas or full blown aneurysms?

• Would this group of drugs make already enlarged, bulging aortas even worse, more dangerous, more quickly? 

Medical Papers in 2015  and the aorta.

In November 2015, two papers were published in medical journals by doctors in Taiwan and Toronto, Canada. Then in May 2016, the FDA issued a new warning.

Here is the abstract of the paper from Taiwan:

Risk of Aortic Dissection and Aortic Aneurysm in Patients Taking Oral Fluoroquinolone

Here is the paper from Toronto:
Fluoroquinolones and collagen associated severe adverse events: a longitudinal cohort study

This paper gives details about what happens to the collagen in the aortic wall:

" In the aortic wall, type I and type III are also the dominant forms of collagen,12 thereby suggesting that a medication contributing to tendon ruptures could also lead to aortic aneurysms. Indeed, pathological sections of aortic aneurysms and aortic dissections demonstrate abnormalities of collagen content, concentrations and ratios.13 Although aortic aneurysms typically develop slowly, our data suggest that fluoroquinolone prescriptions can contribute acutely to aneurysm progression and rupture."

Aneurysms Grow and Rupture More Quickly

Please note that the last sentence, which says that these drugs cause the aneurysm to grow quickly "acute .... progression" and then rupture.  So the general thought that aneurysms do not need to be checked that often, because they grow very slowly, may be dangerous for someone who has taken these drugs.

FDA Warning May 2016

Once again, here is the FDA warning from May, 2016: http://www.fda.gov/Drugs/DrugSafety/ucm500143.htm

 

Here are the first two paragraphs:

"The U.S. Food and Drug Administration is advising that the serious side effects associated with fluoroquinolone antibacterial drugs generally outweigh the benefits for patients with acute sinusitis, acute bronchitis, and uncomplicated urinary tract infections who have other treatment options. For patients with these conditions, fluoroquinolones should be reserved for those who do not have alternative treatment options.

An FDA safety review has shown that fluoroquinolones when used systemically (i.e. tablets, capsules, and injectable) are associated with disabling and potentially permanent serious side effects that can occur together. These side effects can involve the tendons, muscles, joints, nerves, and central nervous system."

While mentioning tendons, which are high in collagen, this statement does not mention the aorta. The papers from Taiwan and Toronto do.

Talking with Doctors

Taken together, these three references help me when talking to doctors about my own family. They help me remember that I should tell them if I or a loved one has ever taken one of these drugs. It also helps me ask for the safest possible drug, if I or my loved one should need an antibiotic. I hope this information helps us all. 

Staying informed together,

Asking our questions,

We are all 

Creating a Climate of Hope.

~ Arlys Velebir

                          Bicuspid Aortic Foundation

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

My Love Will Find You - Families with Bicuspid Aortic Valve

This is for everyone who loves someone with Bicuspid Aortic Valve or any form of aortic disease in the chest.

Derek Owens' parents were told about his BAV at birth
He was 16 when he first had surgery

This is especially for Mothers. Mothers of all ages.  Right from the start, they know how special, how talented their child is. At some point, they may learn there is something not quite right inside the heart of their beautiful child. They agonize through the doctor visits, the surgeries. Some Mothers hear those most dreadful words. Their beloved child is gone. Rest assured, there is nothing you could have done to prevent a bicuspid aortic valve. Above all, you give your child what no physician ever can. You give them your love. It is the most wonderful treatment in the world, and you can increase the dose at any time without harm. Yes, it is the greatest healer there is. Your love. Unlimited. Always.

Following are the words of a childrens' book by Nancy Tillman. The pictures are from BAV families.

Wherever You Are, My Love Will Find You

By Nancy Tillman

I wanted you more

 than you ever will know,

 so I sent love to follow

 wherever you go.

It's high as you wish it. It’s quick as an elf.

You'll never outgrow it... 

it stretches itself!

So climb any mountain...

BJ Sanders at Machu Pich

climb up to the sky!

My love will find you.

BJ's dear Mother, who lovingly
follows her daughter's adventures

My love can fly!

BJ celebrating her birthday in 2014. She had surgery a year earlier.

Make a big splash! Go out on a limb!

My love will find you. My love can swim!

Scott Nichols (in blue) was lost to aortic dissection, January 2014

It never gets lost, never fades, never ends...

if you're working...

or playing...

or sitting with friends.

You can dance 'til you're dizzy...

paint 'til you're blue...

There's no place, not one,

that my love can't find you.

Derek Owens has always loved basketball!

And if someday you're lonely,

or someday you're sad,

or strike out at baseball,

or think you've been bad...

just lift up your face, 

Derek in high school

feel the wind in your hair.

Derek Owens and his Mom, Laura

That's me, my sweet baby, my love is right there.

Chuck Doherty lost his life in April 2012
 due to BAV complications

In the green of the grass... in the smell of the sea...

in the clouds floating by... at the top of a tree...

in the sound crickets make at the end of the day...

“You are loved. You are loved. You are loved,” they all say.

Carrie Mettler running a half marathon

Carrie Mettler's beloved Mother, who knew the reality of
having two daughters with BAV

My love is so high, and so wide and

 so deep, it's always right there, even

 when you're asleep.

So hold your head high

 and don't be afraid

Bob Gies following his surgery in 2010.

to march to the front

 of your own parade.

Doug Grieshop on his wedding day 

Doug Grieshop at 2 months

If you're still my small babe

 or you're all grown,

my promise to you

 is you're never alone.

Doug had an undiagnosed BAV. He lost his life to aortic aneurysm
 rupture 10 years ago, on September 20, 2004.

You are my angel, my darling,

 my star... 

and my love will find you

wherever you are.

You are loved.

Scott Nichols - Bicuspid Aortic Valve and Dissection

Scott Nichols with his son, Carson, on the golf course

Words do not come easily when trying to capture the essence of Scott Nichols. In the prime of his life at age 41, he was a husband to his wife Jennifer, father to his daughter and young sons, friend to many, a special education teacher, and a coach.

Scott coached a high school hockey team as well as being the goalie coach for the NAHL Port Huron Fighting Falcons. He had a lifelong love of sports and continued to play many himself - hockey three times a week, softball, and golf.

Yes, Scott was a very special man, known to so many in his community and state.

There was something else special about Scott. Something that no one, not even Scott himself, knew. He had been born with a bicuspid aortic valve.

A Snow Day in Michigan and Chest Pain

Even in Michigan, where those who live there are prepared for winter, some days the weather is just too severe, and schools are closed. These "snow days" are unexpected free time for students and teachers alike. On a snow day in late January, 2014, Scott Nichols made the most of this unexpected gift of time, packing it with activity. He played basketball with some of the other teachers, and then went on to coach two different hockey teams. Later that evening, he was out on the ice playing hockey himself when he felt the chest pain. 

No Prior Warning

There was no warning that this strong, active man had anything wrong until chest pain forced him off the ice and to the local emergency room. There his heart was checked, and a heart attack was ruled out. The next day his bicuspid aortic valve (BAV) was found. He continued to get worse. What was happening inside Scott? More testing found it. Like many with BAV, Scott's aorta above his BAV had been bulging dangerously (aneurysm). The weak, bulging tissue had torn (dissection). Scott was bleeding inside.

Life Flight to University of Michigan

Scott needed to reach a medical center equipped to perform emergency surgery on his aorta. He was flown to the University of Michigan in Ann Arbor. There, as his family waited, surgeons battled for 14 hours to save him, repairing the terrible injury inside him. But the damage was too great. It had been too long without blood flow to vital places inside him. On January 31st, Scott Nichols was declared brain dead.

Disbelief, Shock, Mourning

Scott (in blue) at the golf course with friends

Scott's family, students, fellow teachers, and friends all descended into shock and deep mourning. This article from the The Voice, February 3, 2014, Scott Nichols Impact Felt, describes the anguish and tremendous sense of loss. As mentioned in the article, with hearts broken the Port Huron Hockey team decided they would play their game the next day in his honor. In the words of Coach Pionk, " ... people like Scott Nichols don't come around very often."

September is Chest Aneurysm and Dissection Awareness Month

The Bicuspid Aortic Foundation observes Chest Aneurysm and Dissection Awareness Month in September.  This same month is observed for brain aneurysms, which also occur in some individuals with BAV. This year, we are deeply grateful to Jennifer Nichols for bravely sharing what happened to her husband just a few brief months ago. She does so in the hope that it may save the lives of others. BAV is estimated to exist in 1 of every 50 people, predominantly in males. 

How many other athletic men in their prime

 are at risk from a bulging, weak aorta?

We do not know. 

We do know that finding a BAV is a red flag 

• to look further 
• to find the hidden danger 
• and to have surgery before a life-threatening emergency.

We hope that through the tragedy of what happened to Scott, the life of someone reading this will be saved. Maybe someone strong and healthy like Scott. Someone who feels great, but gets his heart checked, and finds out he was born with a bicuspid aortic valve. 

Link to Scott's obituary and tribute wall

In raising awareness

of the risk and the danger,

we  seek to create

a lifesaving

 climate of hope.

From all who volunteer

with 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