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 

The Torch is Passed - Dr. Randall B. Griepp, 1940 - 2022

 

Dr. Randall Griepp in 2005

Bicuspid Aortic Foundation Honors Dr. Randall Griepp

The Foundation's first Distinguished Scientist Award was presented to Professor Randall B. Griepp, MD, at a dinner in his honor on April 10, 2005 in San Francisco.

Dr. Griepp's numerous pioneering contributions to the surgical treatment of aortic aneurysm and dissection in the chest have greatly advanced the ability to address even the most difficult aspects of these conditions.

His ground breaking paper on the aortic arch in 1975 opened the door of hope to those patients with arch aneurysms.

In addition to providing aortic surgical training to many residents, Dr. Griepp has been the program director of the Aortic Surgery Symposium for many years. Since its inception this Symposium has been invaluable, providing an international surgical forum focused solely on the treatment of the thoracic aorta.

The Bicuspid Aortic Foundation is deeply grateful to Dr. Griepp for his tremendous contributions in the United States, and through sharing his knowledge with other surgeons, touching the lives of those with Thoracic Aortic Disease through out the world.

The above is shared here from the BAF website.

September 2022 - The Torch is Passed

Dr. Griepp carried the torch of light and hope with courage and humility, fully devoting his many talents to pioneer life-saving surgeries in the face of formidable cardiac and thoracic aortic diseases.

As of this writing, his published work spans approximately 50 years, encompassing heart and thoracic aortic disease surgery. (1)

It is for others now, to carry the torch high, without wavering.

May they be inspired to do so with humility, integrity, scientific rigor, wisdom, skill, and courage, carrying high the flame passed on to them. 

With deepest gratitude

to Dr. Randall Griepp

 and heartfelt sympathy to his family, 

The Bicuspid Aortic Foundation

1) The two earliest and most recent publications for "Griepp RB" in PubMed, as of this writing: 

Needle aspiration biopsy technique in pulmonary disease. Application to therapy.

Castellino RA, Goldstein HM, Stinson EB, Griepp RB.

JAMA. 1970 Jul 20;213(3):463-4.

PMID: 4912737 No abstract available.

A two-year experience with human heart transplantation.

Griepp RB, Stinson EB, Clark DA, Shumway NE.

Calif Med. 1970 Aug;113(2):17-26.

PMID: 4950508 Free PMC article.

Discussion.

Griepp RB.

J Thorac Cardiovasc Surg. 2020 Jun;159(6):2443-2444. doi: 10.1016/j.jtcvs.2019.06.133. Epub 2019 Sep 26.

PMID: 31564540 Free article. No abstract available.

2) Link to landmark 1975 Paper on Aortic Arch Surgery

Prosthetic replacement of the aortic arch

September Awareness 2022 - Thoracic Aortic Disease - "Gradually, then Suddenly"

"Gradually, then suddenly." 

- The Sun Also Rises,

 Ernest Hemingway, 1926

What is happening in the walls of our largest artery, the aorta, gradually and silently, until one day, suddenly, there is a crisis? What about the pressure on a weakened aortic wall? 

My thoughts, as this September Awareness 2022 begins, are with a woman who lives in the eastern US.  She began this year deeply grieving the sudden death of her husband, reviewing his medical background, and bravely seeking answers, thinking of others in their family.

Sudden Aortic Death

It is an unspeakable tragedy when a long and happy life journey together ends without warning, without even a moment for any last expressions of love. 

Early in the morning, she heard the normal sounds of her husband arising, the routine start of a new day. But then, there was a great noise, the sounds of something falling on the stairs. She rushed to see what was wrong.

He never called to her, he never said her name. With her first glimpse of him, collapsed below her at the bottom of the stairs, she heard a little moan. By the time she reached him, he was already gone.

There were paramedics, but then instead of a rush to the emergency room, the wait for the medical examiner to come to their home.  Yes, he is among those who never reach a hospital alive.

The medical examiner called the next day to tell her what was found by autopsy: death was due to natural causes. What were those natural causes?  Cardiac tamponade due to aortic dissection. His heart could no longer beat, compressed rapidly into stillness by his own blood filling the sack around it.

Athletic,  Still Active and Youthful at 80!

Throughout his life, he was healthy and very athletic, excelling in rigorous sports. He remained very active and engaged in the community in his 80th year.

He had been seeing a cardiologist for about 6 years, because of his aorta.

They were told that he did not have an aortic aneurysm, "just an enlarged aorta". His wife interpreted that as "don't worry". He faithfully had annual visits with the cardiologist over those last 6 years.

After his death, his cardiologist told his widow that his aorta had only grown one centimeter in 6 years, and was not that large. He also mentioned his age as a consideration in terms of any intervention.

Some Details

He had a trileaflet aortic valve. He had a "funnel chest" that was never corrected by surgery. There is a bicuspid aortic valve in his blood relationship. 

What about his blood pressure? He was taking one blood pressure medication. He took blood pressure readings at home for awhile, and then later stopped.

What was his blood pressure during the days preceding and on the morning of his aortic crisis?

When the Thief Strikes

I hope it was some comfort to spend time with me, for her to tell me about this wonderful man so abruptly stolen from her. I think of her, together all those decades, coping through those first days, weeks, and now months without him.

There are many aortic specialty centers in that part of the United States where this family is. She cannot go with him for another opinion. The cruelty of this disease has done its worst.

She can, however, encourage their family, his living blood relatives, to seek out evaluation and care at one of them. Sadly, they now have a family history of a fatal aortic dissection to list on medical forms, due to the loss of a vibrant man, still youthful regardless of his number of birthdays. 

Yes, they know now that no matter what it is called, "enlarged aorta" or "aneurysm", it can kill. 

May rigorous pursuit of answers

prevent thoracic aortic disease

from gradually,

 then suddenly,

doing its worst.

~ Arlys Velebir

                         Bicuspid Aortic Foundation

September TAD Awareness 2021and Life-Long Aortic Care

 September - A Month for the Aorta!

Today BJ Sanders 
 Lives her Best Life Story
 through Proactive
Life-Long Care 

Since 2005, BAF has observed September as  Awareness Month for the aorta in the chest.

 Although those who have bicuspid aortic valves and their family members have a great deal of interest in the heart, whose month is February, we have learned that the aorta is vitally important also. We need them both to receive care to live our best life story! We also know that still today, Thoracic Aortic Disease (TAD) is too often overshadowed by the heart or just ignored altogether.

This September, BAF remains convinced that the aorta is more than worthy of  it's own month, distanced from February and "Heart Month" to avoid confusion.  At BAF we are delighted by the various days and weeks also designated this September that focus on the aorta! The aorta needs all the global attention it can get! 

This year BAF particularly highlights living your best life story through proactive Life-Long Aortic Care.

Life-Long Care and the Aorta

BJ Sanders is among those with BAV/TAD who receive Life-Long monitoring and care, including follow up imaging for both her heart and aorta. Described by one cardiologist at a major center as "complicated", she knows the importance of being proactive in order to achieve her best life story. 

The Aorta is Not the Heart! 

First of all, let's get better acquainted with a vital part of us that we may not have heard much about - our aorta! What is it, and how do various diseases affect it?

Diseased aorta in the chest

While there are some articles that describe the aorta and aortic dissection in the press, the aorta and the heart are sometimes blurred, potentially confusing the reader. Much more concerning and dangerous to life itself is confusing the heart and the aorta in a medical setting, often an Emergency Room(ER).

When doctors say our pain is not from the heart, we can ask them to check the aorta. In the United States, CT imaging is widely available and will find aortic aneurysm/dissection.

Very often there is no second chance to prevent an aortic catastrophe.

 At BAF, this September we do not forget Doug Grieshop and his family. This September19th would have been Doug's 50th birthday.

Doug Grieshop - Forever 33

 It remains particularly hurtful that, having checked him for signs of "heart disease" and failing to look further (at his aorta)  for a reason for his chest pain, this strong young man was given anxiety medication, which he took faithfully including the day his aorta ruptured. It was an autopsy that told the truth about his aorta, previously scarred by a small tear and now fully ruptured, his life story had ended much too soon. The diseased aorta remains among the subset of vascular diseases most frequently contributing to serious injury and death.

The Aorta is Not a Tube!

The authors of this recent paper from Italy begin by introducing the aorta:

Pathology of the Aorta and Aorta as Homograft

They open with "The aorta is not a rigid tube, it is an 'organ'....", and go on to beautifully describe it.

The aorta is not "just" a tube, any more than the heart is "just" a pump. The aorta is a vital entity in its own right, and prone to various disease conditions, as these authors describe.

We continue to press for the aorta to be included in proactive diagnosis and ongoing care for all those who need it so much. In a series of articles throughout September, we will share examples of those who would benefit from and need Life-Long Aortic Care. Sharing information is an avenue to empowering the public to advocate for their own best care.

TAD is often silent, taking years to suddenly explode into a bleeding emergency in the chest. Our theme is simple this September:

 Receiving Life-Long Aortic Care

we can

Live our best life stories,

 Creating a Climate of Hope, 

~ Arlys Velebir, Bicuspid Aortic Foundation

Pursuing Answers in Memory of Those We Have Lost - Heart Month 2021

Red Jasper

 On this first day of February, designated as Heart Month 2021, at the Bicuspid Aortic Foundation we begin by remembering those we have lost, for it is in remembering them that we are confronted by one of the most compelling reasons we exist, the persistent, tragic loss of life, too often in the young. Despite advances in diagnosis and treatment, we continue to lose them. We begin by remembering some young men in their 30's.

Forever 33, Almost 34, in 2020

Michael Anthony Smith was born in August, 1986. Just under a week before his 34th birthday, in August 2020, he passed away in a health center in Gainesville, Florida. 

Michael's memorial page, including a video tribute, are located here. 

Those who visit the link above and watch the video will recognize a strong, active, vibrant young man. There are no words to adequately convey the tragedy of his loss, the pain and emptiness of those love him.  

Forever 33 in 2004, Forever 34 in 2012, Forever 30 in 2013

Over the years we have heard of other young men who succumbed unexpectedly to BAV/TAD complications. Doug Grieshop turned 33 the day before his death in 2004; an autopsy revealed a ruptured aorta and an undiagnosed BAV.

Chuck Doherty was 34 that day in 2012 when he collapsed in the driveway of his home as he returned from a run; Chuck's BAV and aortic stenosis had been diagnosed, but that was insufficient to protect him from sudden death.

Chad Rogers was 30 when he failed to return from his run on Monday evening, July 22, 2013. His body was not found until Friday afternoon. Chad's BAV was known since infancy, but similar to Chuck, his physical activity had not been restricted. 

Doug Grieshop's family shared his life initially here, and there is more in this follow up, Forever 33.

Chuck Doherty is remembered here.

An account of Chad Rogers' cause of death is here.

There are others, also young, also athletic, also suddenly taken, whose families have come to us after their loss. As I type this, I remember an autopsy report sent to BAF years ago; it described the examination of a healthy young man's body. He had dropped dead while playing, if I recall the sport correctly, volleyball with his friends. The report detailed his body, healthy and normal with just one exception. The only finding was a bicuspid aortic valve; despite CPR immediately, his heart could not be restarted, his death perhaps due to an abnormal heart rhythm. 

And so we begin Heart Month 2021 remembering this group of young men, healthy appearing, athletic, vibrant, often in their 30's. 

It is approaching 17 years since Doug Grieshop died, and in the intervening years other young men in their 30's have died also. How many, we do not know. How can we see beyond their glowing, healthy appearance and identify those at risk of a deadly event (aorta, aortic valve, arrhythmia) before they are prematurely taken, leaving loved ones to go on somehow, deprived of their gifts, energy, and  many contributions to our world?

In their memory, we pursue the answers,

in February 2021, 

Creating a Climate of Hope,

~ Arlys Velebir

Bicuspid Aortic Foundation

TAD Awareness September 2020 - More Awareness, Many Challenges Today

On this last day of September 2020, where are we in terms of awareness of aortic disease in the chest, both for the public and for medical professionals? 

Are We Still Learning "the Hard Way"?

Once the aorta threatens or takes away someone's life there is painful "awareness". It is a "hard way" to learn about a potentially deadly condition. For physicians, missed or delayed diagnoses and possibly loss of a patient are also learning "the hard way"; that is, if the diseased aorta is actually ever discovered at all. It is possible, in the absence of autopsies, that deaths due to aortic disease in the chest remain hidden, uncounted.

Would it Be Different Today?

At the time Michael Kirk went to the ER with symptoms, he was a cardiac intensive care nurse at that very hospital.  No one thought of his aorta, although over an agonizing 68 hours, they thought of many other things, including anxiety! The medical team ultimately learned from Michael, and he survived the crisis.  Michael shared his experience with BAF here. Thinking of Michael's experience, would it be better in 2020? Would his family members, assumed to have died suddenly of "heart disease" be checked for aortic disease today?

In 2020, Someone Still Needs to Think of the Aorta in the ER

There is a marker in the blood, troponin, that indicates heart muscle injury (typically signs of a heart attack) that appears to be broadly understood and used. There is no simple equivalent for the aorta today. Currently, someone needs to think of the aorta, to rule it in or out with imaging, as one of the major killers in the chest.

 However, a paper published in July 2020 offers hope for additional diagnostic help in the future. Imaging and Biomarkers in Acute Aortic Syndromes: Diagnostic and Prognostic Implications :  "It is expected, in the near future, the development of serologic and imaging biomarkers able to early detect clinically-silent pathologic changes in the aorta wall before (primary prevention) and after (secondary prevention) the acute index event."

This is hopeful news! However, checking for these biomarkers would still require that someone thinks of the aorta, not just the heart!

This court case is an example of death after being sent home from the ER, when the aorta was not considered. 

Richard Houchin, whose aorta dissected in 2004,  remembers hearing someone say "aorta" while in the ER, and thinking fuzzily, what is my aorta? Richard had a number of things "go right" that day, beginning with the paramedics who thought of his aorta, despite instructions from a major medical center to treat him as a heart attack victim.

When it is not a heart attack, we can help physicians think of the aorta in the chest by bringing it up to them.

In 2020, Someone Needs to Care for Aortic Dissections Survivors

An article published this September has this poignant title: 

Am I going to die now? Experiences of hospitalisation and subsequent life after being diagnosed with aortic dissection

The full article is not freely available to the public. However, as the abstract indicates, this is an effort to understand and improve the experience of those who survive aortic dissection. 

In 2020, Someone Needs Answers and Support When Thoracic Aortic Disease Causes Sudden Death

There are no words to describe the devastation left behind among the living who suddenly lose a loved one. 

How many times is aortic disease in the chest the actual culprit, not a "heart attack"? 

Amy's family had the courage and resources to arrange for a private autopsy, which unmasked the undiagnosed BAV and diseased aorta that abruptly took her life. Here is Amy's story.

In 2020, Focus is Still Needed on the Aorta, Not Just the Heart

For those with BAV, they may only be told about their aortic valve and heart. I recently spoke to the mother of a wonderful, active boy, age 14. While hospitalized at a major medical center for another reason, the presence of a bicuspid aortic valve was discovered. The initial information given was not overly concerning. When following up with a local cardiologist later, however, this boy and his parents were shocked to be told that his aorta was enlarged, and his physical activity should be limited. 

How could that be possible? His aorta had not even been mentioned, and all of a sudden, it is an issue important enough to restrict his sports participation? This is devastating to anyone, let alone an active, strong teenager.

It was not easy in my own family when a large ascending aortic aneurysm was "discovered", years after being told all was safely fixed following BAV replacement surgery. Later we learned the aorta was already enlarged at the time of the BAV replacement and had quietly continued to grow! 

No, it is not easy to work through the emotions and lifestyle changes that may follow abruptly learning about an enlarged/aneurysmal aorta. Acceptance of physical restrictions and lifechanging adjustments are not trivial for those whose bodies are seemingly so healthy, often naturally gifted athletes who are typically involved in numerous activities and sports. 

Parents, families, and each one with BAV deserve compassionately shared accurate information about their aorta along with their BAV/heart, ultimately enabling them to live with confidence, supported by their medical care team.

The following paper published in 2020 provides international information from multiple medical centers, regarding 2,122 BAV children; half of them had an enlarged aorta. Their average age was 10.2 years.

Predictors of Bicuspid Aortic Valve-Associated Aortopathy in Childhood: A Report From the MIBAVA Consortium

Keeping Our Focus on the Aorta Every Day, Every Month, All Year Long

At BAF we have been observing September TAD Awareness for a number of years. We find in 2020 that the aorta in the chest still presents many challenges. The various efforts to raise awareness this year all help the public understand the importance of their aorta, this large candy-cane shaped artery that begins with their heart. May we maintain this focus every day, every month, all year long. 

 On this last day of September,

 we can help everyone we meet

to remember the aorta and

seek accurate information,

empowering them to live confidently,

 Creating a Climate of Hope,

~Arlys Velebir

                         Bicuspid Aortic Foundation

Thoracic Aortic Disease Awareness September 2020 - TAD in the Midst of COVID-19

 How many have been injured or died from a diseased aorta in the chest (thoracic aortic disease (TAD)), not receiving treatment for various reasons during the COVID-19 pandemic?

Did the pandemic come between those with a torn aorta in the chest and the emergency physicians and skilled surgeons who could save them? It is reasonable to believe it did. The following is written by physicians in New York:

The COVID-19 Pandemic and Acute Aortic Dissections in New York

A Matter of Public Health

In the above, a "significant and precipitous drop" was identified in the number of surgeries for acute aortic dissection. The best hope for someone with acute type A dissection, which involves the ascending aorta and strikes without warning, is coming to the ER, being diagnosed, and receiving emergency surgery. How many were denied that opportunity due to the pandemic?

As the above concludes:

"It is critical, as we adjust to the pandemic, to balance the public health imperative of social distancing with the individual need to consult in the presence of sudden severe symptoms. Furthermore, additional resources, ranging from telemedicine to numbers of first responders, should be greatly increased. This serves as a word of caution for cities yet to experience a surge in COVID-19 cases, as well as for future similar events."

Inspiration and Also A Warning

The following is an account of a professor from Idaho who traveled to New York for life-saving complex aortic surgery during the pandemic. It is hopeful and inspiring, while at the same time containing a warning about the vital importance of lifelong monitoring of the aorta in those born with bicuspid aortic valves.

BACK IN BUSINESS: CEED Director undergoes life-saving heart surgery in New York amid pandemic

One of the surgeons who saved Jeff Street, pictured with him in the article, is Dr. El-Hamamsy, who is also among the authors in the first article above. He is among the skilled surgeons that could have helped those living nearby also, if only they had reached him.

Regarding the warning in this man's experience, it is important to avoid unnecessary, life-threatening aortic dissection or rupture in the first place. How did this man's aorta reach this very fragile state undetected? 

Jeff Street had surgery to address his bicuspid aortic valve 23 years earlier. Presumably he had cardiac care over those years, but what about his aorta?

Only when he became symptomatic this year, the article states, was an aneurysm "discovered". More concerning still, was the actual condition of his aorta when fully exposed during surgery on June 9th (quoting from the article):

" “Absolutely it was a life-saving surgery,” Street said. “I did not know that my aorta already had a small leak on it. The only thing holding it together was the scar tissue from my previous surgery 23 years ago.”"

Words like "miracle" come to mind as one reads about this man's experience - fragile tissue somehow holding together until he was in the hands of a surgeon with the skills to save his life.

And then, thoughts turn to those whose lives have ended abruptly, unknown and uncounted.

For those in BAV families, aortic valves are unquestionably important and vital. It would be beyond a tragedy to provide state-of-the-art treatment and care for their aortic valves, but not their aortas. 

This September 2020, once again it is important to raise awareness of the aorta in the chest in those born with bicuspid aortic valves and their family members.

In the midst of  this pandemic,

We again highlight the aorta in the chest, 

Raising awareness

and  

 Creating a Climate of Hope,

  ~Arlys Velebir,

                           Bicuspid Aortic Foundation

 

 

 

The Power of Hope and "Redo" Aortic Surgery

Introducing Joseph
It was 10 years ago. We were two voices on the phone together. We have never met in person. I have never seen his picture. None of that matters, not then and not now. What mattered was what we said to one another.

His name is Joseph. Heart surgeons refer to someone like him as a "redo".

His daughter had put us in touch. He was already a survivor of an aortic crisis, having come through aortic dissection and ascending aortic aneurysm surgery. Now his bicuspid aortic valve had become calcified and narrow. He was facing another major open-heart surgery, not so very long since the last one.

Yes, surgeons call someone like Joseph a "redo". I had heard some things about "redo's", such as the challenges of scar tissue that can make even opening up the chest safely a challenge.

An example of BAV Stenosis
This was not Joseph's valve!

How could I help Joseph? 
By this time, I had already been through enough in my own family to know about facing hard things, including more than one open-heart surgery, because of BAV and the aorta.  I also knew how much strength and ability to heal those with BAV often have. They are generally not "frail" people, and as Joseph told me about himself and how active he was, I recognized that strength in him.

He had already come face to face with death when his aorta tore. He had come through major surgery to replace part of his aorta. Now it was his stiff, calcified BAV forcing intervention. Before open heart surgery and artificial aortic valves, the end state of aortic valve stenosis was heart failure ending in death. There was no question the stenosis could not be allowed to weaken his heart and end his life, when a surgeon could help him. He already knew these things from his doctors. I must leave to them all the challenges of a "redo" surgery.

Joseph didn't need information from me. 

He didn't need any explanations either.

He needed hope.

"Am I going to make it?"
I am so glad that Joseph felt comfortable asking me this, the life-and death question that weighed heavily as he chose to have surgery again:

"Am I going to make it?" 

Across the years, I hear once again the sound of his voice as I write this.

"Yes, you are! And we will talk again!"  

And talk again, we did! 

What a moment, hearing his voice again!

 Yes!!!

There is nothing like it! 

Courage

Red Jasper
The Warrior Stone of
Strength and Courage

Fear is normal; we all have fears to face when entering a hospital and having such major surgery. Courage enables us to go forward, despite our fears, and receive the help being offered. Joseph was a very courageous man. It was my privilege to support his courage, knowing that others had come through multiple surgeries like this.

I have sometimes used the analogy of surgery being a doorway or a gate; something we go through and there is our life once again, the diseased valve or aorta that plagued us left far behind.

A decade has passed. Joseph was not a young man back then. Age in itself is not a limitation to receiving help! How beautiful to think of  him continuing forward and having the precious time since then.

Hope
Thinking about Joseph today inspires and moves me to share this experience with others. There are others facing yet another surgery because they were born with a bicuspid aortic valve; others needing hope and support of their courage today.

Perhaps I should mention that my late husband had a total of four open heart surgeries over 27 years. Four precious times when I could keep my promise to welcome him on the other side of the surgery door, as he awoke from anesthesia.

There is so much that is possible today, while we look forward to an even better future. TAVR technology has continued to improve, emerging as an option to be individually considered in cases of both native and prosthetic aortic valve stenosis. While we must continue to speak up about our needs and the things that must be improved, this hopeful "climate" surrounding us is central to the Bicuspid Aortic Foundation's mission.

Joseph and I are sharing

this especially with you,

As you bravely choose and prepare for

 another surgery,

 "Creating a Climate of Hope".

~ Arlys Velebir

                           Bicuspid Aortic Foundation

Aortic Dissection - They Cannot Find What They're Not Looking For

Diseased aorta in the chest, before dissection/rupture

There is aneurysmal enlargement (root and ascending),

 abnornal lengthening(elongation)

 and curving (tortuous)

I receive a daily alert on certain key words, including "aortic dissection". Recently, the headline on one of them was "Calif. Hospital Preserves Win in ER Patient Death Suit". Reading the published court proceedings, I was heart broken. A man was dead, sent home from an Emergency Room with an undetected aortic dissection, and the headline was about a hospital winning... legal winning, yes, but in terms of human life, it was only loss. 

When an aortic dissection is not found in life,  if there can be any so-called "winner" at all, it is the disease itself.

Why is aortic disease, in its most deadly state,

still not being diagnosed?

Through the courageous and persistent efforts of Jennifer Simon, following the failure to find aortic dissection in the ER where her husband Ken died, I heard about changes that were made and how others with aortic dissections were diagnosed through her efforts there.  Jennifer spoke about what happened to Ken here. As Jennifer says in the video, "They cannot find what they're not looking for".

Ken Simon died in Washington state in 2003.

 John Ritter died in southern California that same year.

They both made it to a hospital.

In June 2020, 

 I am reading about another man,
 who also made it to a hospital.

 His dissection was not found during the hours he spent there.
Why not?

Dissection was a word I first read in medical papers as I struggled to understand the aneurysm in my late husband's chest in 2001. It wasn't until September 2003, while driving one morning, that I heard a news report saying John Ritter had died of an aortic dissection. It was the first time I heard the word dissection used to inform the public. I remember exactly where I was when I heard it. That will soon be 17 years ago, and yet in June of 2020, I am reading an announcement of  a hospital winning (again) a lawsuit on appeal. A lawsuit about a man who came to their emergency room at 4:03 AM and was later discharged, leaving at 11:16 AM. When he left, he had less than 8 hours to live; not nearly long enough to follow discharge instructions to see his doctor and/or a cardiologist the next day.

At that moment, no one knew it, but aortic disease had already won.

All else was lost.

The opportunity to save a man's life was lost.

All hope of this man having emergency aortic surgery was lost.

Ultimately his life was lost.

His family lost precious time, their future, 

 with their husband and father.

The tearing of the aorta is the critical end state of disease
 in this blood vessel.

There is still hope for life to win, through surgery,

as long as the strong outer layer of the aorta holds together, 

but only if the dissection is found.

Reading the court proceedings, remembering my own visits to ER's with my late husband for various reasons, I recognize what was done: asking questions, taking vital signs, chest x-ray, EKG, multiple blood draws (including looking for the enzyme that indicates a heart attack, troponin). A heart attack likely would have been found, but that is not what was happening. No CT scan, no ultrasound tests were done. A cardiologist was not called. They did not find his torn aorta.

 As I recall reading the public information about John Ritter, he had a chest x-ray too, but not a CT scan. CT scanners were and are widely available, typically located very near the emergency department, since this test is both so fast and so useful. Among the many virtues of a CT scan, it makes a torn aorta visible to a radiologist.

Many years apart, in different hospitals, I suspect that both of these men were approached similarly in the emergency setting. They did not have heart attacks. Despite the many medical papers published, guidelines written, and efforts to raise awareness about aortic disease in the chest since 2003, many years later another man still wasn't given a chance to have surgery, a chance to at least fight, along with surgeons, for his life. 

CT and Triple Rule Out of the "Big Three" Killers in the Chest:

 Heart Attack, Aortic Dissection, Pulmonary Embolism

 Perhaps there are many reasons for the chest pain that causes someone to seek emergency care. The medical literature, including this paper from Ottawa, mentions the use of CT to find the three most common immediately life-threatening conditions that cause chest pain: heart attack, aortic dissection, and pulmonary embolism.

I do not understand why it would be hard to remember and to rule each of these three deadly events either in or out. Perhaps it is not so much memory but focus that is the issue. Blood test results may quickly show that the heart muscle is not damaged - that there is no indication of a heart attack. If it's not a heart attack, why not check the aorta?

It is very hurtful for me to read, in the court record, the medical history of this man, documented from a prior hospitalization. He had a heart murmur. He had high blood pressure. He had a history of smoking. Even prior hernia surgery gets my attention.  There is no record of anyone ever connecting any of those dots regarding aortic disease. One of the issues raised in the court proceeding was that this existing medical record, apparently available, was not reviewed when he was in the ER. Rather the ER notes say that medical history was provided by the patient and there was no past medical history. He was also described as being overweight and having high cholesterol.

That morning in the ER he may have appeared to be a candidate for what is commonly called "heart disease", coronary artery disease, which is the leading cause of death. It is indeed important to find a heart attack, of that there is no doubt. When a heart attack was not found, what about the other deadly killers in the chest? What about the aorta?

The court document mentions his symptoms in the ER.  It is beyond sad to read what he was feeling as the chaos caused by  aortic dissection disrupted vital blood flow in his body. A radiologist's description of his chest x-ray is in the court document too, mentioning a mildly enlarged heart. Again, I wonder about his heart murmur. 

Hope from Those Who Continuously Seek to Improve 

I found some hope in a new paper available here (June 1, 2020) about a 65 year old woman who initially had chest and abdominal pain, but upon arrival at the emergency department, the pain was in her leg. She had an aortic dissection; the paper says her dissection was found, she was transferred to another hospital for surgery, and recovered well.

Despite successfully saving her, the writers (all listed as working in Emergency Medicine) discuss the focus on her leg pain initially, and the importance of the medical history, from both the family and the emergency medical personnel, so that important information, such as the original chest pain, is not missed. The use of ultrasound as an alternative to CT to find the dissection is also mentioned. I am encouraged by their focus on continuously learning and improving in this paper, so that more people may receive the correct diagnosis and are sent for surgery as quickly as possible. The passage of time is on the side of the disease, not the fight for life. 

In the ER with Chest Pain 

Writing this, Doug Grieshop is on my mind and in my heart. He is the first person, killed by aortic disease, that I came to know through his family after his death. Like so many with BAV, he was energetic and very active; he looked so vibrant and healthy on the outside. He was a loving husband and father, in the prime of life. You can meet him here: Doug Grieshop - Forever 33  Between the time he went to the ER with chest pain and the day he suddenly died, many months went by. There was time, missed opportunities to find his BAV/aortic disease and have surgery. He died almost 16 years ago, in September 2004. His children are teenagers now.

As Jennifer Simon said regarding what happened to Ken,"They cannot find what they're not looking for". Each of us may need to be the one that remembers our aorta, in the presence of chest symptoms.

Think life and death and speak up!

When doctors say it's not a heart attack,

insist that they check your aorta!

For anyone interested, here is the recently published court document. Among other things, it sheds light on the legal view of a patient's written acknowledgement that a physician is a contractor, not an employee, of a hospital.

How Many Deaths Will It Take . . . 'Til We Look at the Aorta?
I close with this link to "Blowin in the Wind" performed by Peter, Paul and Mary, and these haunting words

"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."

There is an answer, broadly available technology,

 to find aortic dissection in the emergency room, 

if only someone will think of the aorta.

An unused CT scanner,

 mere feet away from a dissection victim,

is beyond tragic. 

  Someone should not have to die in order to teach others

 to think of the aorta in the chest.

When that happens, aortic disease has defeated everyone.

How many deaths, how many more years, will it take?

 

When the aorta tears,

swift diagnosis 
is the beginning
of
Creating a
Climate of Hope,

                     ~  Arlys Velebir
                                               Bicuspid Aortic Foundation

Published in 2020, updated April 2021

Questions in Search of Answers in 2018

Questions Without Answers

Why do some BAVers tear/rupture their aorta?

Why do some BAVers have aneurysms that do not tear or rupture?

Why are there BAV/aneurysm and  TAV/aneurysm in the same family?

Why do some BAVs calcify and narrow?

Why do some BAVs leak?

Why do some BAVs do both: calcify/narrow and leak?

Why do BAVers get infection (endocarditis) in their hearts? 
Why do some BAVers develop blood pressure issues?

Why are many BAVers so athletic and energetic?
Why do many BAVers (and their TAVer family members) have "delicate tissue", joints prone to injury, "bad eyes", and other issues through out their bodies?
Why can't my doctor tell me ......?

Why............?

Why............?

Why............?

There are so many unanswered questions when it comes to the individualized care that each person with BAV and their blood relatives should have.

As this year comes to a close, we invite you to support our search for answers. Many of those answers may be found in the mice families being studied at the University of Ottawa. With your help, the Bicuspid Aortic Foundation wishes to continue to support this vital work. Online donations through Razoo may be made here. 


Thank you for joining us in our search for answers.

Best wishes in 2018,

~ 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 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

What Happened to Amy?

This is Amy's story. Only her name has been changed, to protect privacy.



Where is Amy?

"It's just not like her, Amy would have let us know if she couldn't come!" And so a "welfare check" was requested. Her home was silent. There was no response to the doorbell, the knocks, the calls of her name. Something was terribly wrong!

They found Amy inside her home, lifeless. Apparently there had been no opportunity to even call for help.

What Happened to Amy?

Her family members were notified. The police arranged for her body to be taken directly to a local mortuary. Deeply shocked, her adult children needed answers. Their mother had been an active, independent, and apparently healthy woman. She did not even have high blood pressure. They thought she would be with them for many years to come. How could this happen, without warning?

 They needed answers.

In the place where Amy lived, no one offered to find those answers. After all, there was no evidence of a crime, no villain for the police to pursue. The death was clearly due to "natural causes". But, why? Is it really "natural" to die like this, without warning, when someone is so healthy?

Why Did Amy Die?

Amy's daughter told me that not knowing was unbearable to her family, much worse than the thought of an autopsy would be. Despite their tremendous shock at her sudden death, Amy's family found the strength and the financial resources to request a private autopsy. The local medical examiner was not available, and so Amy's family found their way to someone they describe as special, a compassionate, caring physician who carefully examined their mother's body and helped them understand.  What that physician found inside would have been a gruesome sight.

Quite simply, Amy bled to death. Unknown to her, she had an aortic aneurysm in her chest just above a bicuspid aortic valve in her heart. Because there was an autopsy, Amy's death certificate is accurate, stating that death was caused by the rupture of an aortic aneurysm in the presence of a bicuspid aortic valve (BAV).

Courage to Press for Answers

Sometimes families cannot bear the thought of an autopsy on their loved one.  There also may be veiled opposition, if the death occurs in a hospital, from a medical establishment that fears malpractice lawsuits. Sadly, the days of Dr. M.E. Abbott and Sir William Osler, who learned from autopsies and published extensively about aortic disease in the chest, are gone. As with Amy's family, it is the survivors' legal right to have an autopsy, but they may have to pay for it.

Knowledge - a Gift of Life 

Amy's family was left with the sad, painful task of sorting through her things, closing her home. She was an avid reader, and they found many articles about keeping healthy, preventing disease. Among them may have been the usual warnings about preventing stroke and "heart disease". Like many with BAV, Amy's arteries in her heart were perfectly clean. She was not at risk of a heart attack. Sadly, there was nothing about BAV and aortic aneurysm, the condition that suddenly robbed her of life. Amy would not have even heard about what snatched her life away. If her family had not arranged a private autopsy, the reason for her death and the potential threat to their own lives would never have been known by anyone at all.

Knowledge like this comes at an unspeakable price. It is a price too high, too great to pay. It should not happen.  There is a well known medical center not far from where Amy lived that could have performed her aneurysm surgery. They do this surgery frequently, perhaps almost every day.

Amy's blood relatives are educating themselves, being checked for BAV and/or aortic aneurysm. No one needs to tell them why now.

They know.

The Uncounted

The Bicuspid Aortic Foundation remains extremely concerned about the uncounted deaths due to BAV and aneurysm complications. Amy could easily have been one of those uncounted deaths, if her family had not had the courage and ability to seek the truth. Sudden deaths are too often simply recorded as due to natural causes, perhaps assumed to be due to a heart attack, but without any proof. 

The lack of understanding and recognition of aortic dissection/rupture deaths in the presence of BAV, as well as sudden aortic valve related deaths has consequences not just for those who die, but for the living.

Other blood relatives may be at risk. But there are also consequences for the entire BAV population. Because these people are uncounted, they are not found in the statistics, not included in the medical papers published about patients who receive treatment. How can we understand those most at risk, those who may never even reach a hospital, if we don't learn from them? We don't even know why they died! And so, the statistics upon which treatment of the living is based can not include these unknowns, perhaps the most vulnerable, the most fragile. Must they continue to die?


 Who Will Speak for Me?
Amy began this year, 2016, as we all did, with plans and hopes for her future. They were never to be realized, because she had been born with a bicuspid aortic valve, and over time an aneurysm developed. Today her voice is silenced. Who will speak for her, and the others we lose? 


At the Bicuspid Aortic Foundation we can help give them a  voice and bring into focus these most vulnerable. We are enlisting the aid of pathologists to do so.
They are the physicians who speak for the dead, and in doing so, may save the living. We are also encouraging families where sudden death has occurred to take courage and inspiration from Amy's story, and seek the answers they need.

In giving a voice to Amy,
And others we have lost,

May we help the living
And in their memory, 

Create a Climate of Hope.

- Arlys Velebir