Wednesday, September 30, 2020

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

Wednesday, September 16, 2020

September Awareness 2020 - Aortic Follow Up Tests Amid COVID-19 Pandemic

No photo description available.

No matter where we are in our aortic journey, checking on aortic status (aorta, aortic valve, heart overall) inside the chest is part of life, as long as we live. It is so fundamental that it is the "A" in the Bicuspid Aortic Foundation's "Aortic ABC's"

Imaging tests such as echocardiogram, CT, and MRI, provide a great deal of information about aortic disease. Providing amazing detail, state-of-the-art CTA tests provide vital information, including aortic valve leaflet function in the beating heart. 

Serious, life-threatening issues with the aorta in the chest may be silent, nothing wrong is "felt". Imaging studies to check the aorta (and heart) are important in order to prevent potential injury and loss of life.

Following is an account of the tragic loss of a young man whose aortic CT scan was not done in early June as planned, due to a COVID-19 lockdown at the hospital. He collapsed while jogging on August 13th and died August 15th. The CT scan intended to check the status of his aortic stent was never done.  Reading about the damage inside his body that ultimately took his life is indescribably shocking, horrifying. He was only 19 years old.

Corbella: Prairie teen killed by lockdown, not COVID-19


Between a Rock and a Hard Place in 2020
COVID-19 complicated decisions around imaging in my own family this year. Initially all went so well, when someone traveled out of state to receive a TAVR valve along with a stent to protect blood flow to the heart. Yes, highly skilled hands saved a life, just before most medical facilities "locked down". But plans for follow up imaging, both echo and CT, were deferred. And then, when an echocardiogram was available locally, it showed an alarming increase in pressure across the new TAVR valve.

What could possibly be wrong? 
One possibility was a complication called leaflet thrombosis that sometimes happens with biological valves. Blood clot formation, including the potential to block the stent which would be deadly, needed to be ruled in or out. It would take a specialized CTA scan to understand what was happening, only available by out-of-state travel.

Weighing the Risks - the Virus or the Heart?
Sometimes the decisions that must be made can be almost overwhelming. The specialized CT protocol was shared with local medical cardiac professionals, but they could not provide it there. The choice was: risk COVID exposure, including air travel, or risk the new valve and stent. Either one was potentially deadly. Ultimately, the decision to travel was made, taking all possible precautions. 

What happened?
The specialty CTA scan revealed that the TAVR valve and stent were fine, no blood clots! It was explained that sometimes, in a case like this, the echocardiogram is not able to accurately calculate the pressure difference across the aortic valve. Yes, sometimes it is complicated. 

It was good news, but risk was taken to get it. Thankfully, the travelers did not contract COVID-19. This is the reality of dealing with aortic disease in the chest - the aorta and the heart - in 2020. Aortic disease is challenging for many reasons, and the unknowns of COVID-19 have added to those challenges, as it has for other conditions. I have had conversations with others who struggle with going for follow up echocardiograms and cardiology visits. To date, they have done so safely, as hospitals and physicians develop sanitation protocols to protect everyone. There is a renewed emphasis on infection prevention, which is a good thing, for both viral and bacterial infection prevention.

As you and your doctors
follow your aorta and your heart,
in 2020 and the years to come,
you are 
Creating a Climate of Hope,
~Arlys Velebir
                          Bicuspid Aortic Foundation 






 

Tuesday, September 8, 2020

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:

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

 

 

 

Thursday, June 11, 2020

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


Tuesday, June 9, 2020

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


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