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

Misdiagnosis and Disease - A BAV Perspective

Misdiagnosis and the "Big Three" Categories: 75% of "Serious Harms" , 50% from 15 Diseases 

Two papers published in the journal Diagnosis in July 2019 and May 2020 seek to shed light on the harm done by misdiagnosis of known diseases. These open-access papers are available online, as listed below (1) (2).  The authors identify three major categories and five diseases in each of them that are estimated to account for about 50% of serious injury and/or death: vascular events, infections, cancers. 

A Bicuspid Aortic Valve Perspective

Of the three categories, both the vascular and infection disease categories are of particular importance to those with BAV. Clinical significance of the bicuspid aortic valve , published in 2000, states that BAV may be responsible for more death and morbidity than all other congenital heart defects combined. Perhaps it is not surprising then, that the diseases listed include several vascular and infectious diseases known to cause injury or death in some who were born with BAV.

Diseased aorta in the chest

In the vascular events category the 5 diseases are:

• Aortic aneurysm and dissection
• Arterial thromboembolism
• Venous thromboembolism
• Stroke
• Myocardial infarction (heart attack)

In the infection category the 5 diseases are:

• Sepsis
• Meningitis and Encephalitis
• Spinal abscess
• Pneumonia
• Endocarditis

From the authors' conclusion: "We estimate that roughly one in 10 patients* with a dangerous 'Big Three' disease is misdiagnosed, and roughly half of those misdiagnosed die or are permanently disabled as a result." (1)  They go on to note that there is no indication that the error and harm rates are declining. They also mention there has been improvement in those diseases that have received actual sustained emphasis over several decades. 

BAV and These Diseases

Reading these papers I reflected on my late BAV husband's experience with 5 of the 10 diseases above at different points in his life, and the challenges we faced with diagnosis and treatment. He survived these serious threats to his life until intractable bacterial endocarditis ended it. His doctors marveled at how much he was able to overcome, which is the case with so many other "BAVers" I know.

Each person with BAV has their own journey. Seeking help may bring them to a physician's office, the
ER, perhaps both. Once there, even at the very first indication of an issue, will the disease be found in time to prevent disability or death?

What Can We Do? Be Informed, Be Proactive!

What can we do when we need help? We can be aware of these too often-missed diseases - the possible presence of an aortic aneurysm, the pain of an aortic dissection crisis, the potentially deadly infections - sepsis, endocarditis, or even pneumonia.

 With that awareness and realizing that these diseases may be missed, we can ask questions and whenever possible get additional opinions from the best expertise available. 

We can pursue answers,

 without embarrassment when discounted and misunderstood,

realizing the consequences to our future

if potentially deadly conditions are not found in time. 

Perspective from Sir William Osler in 1907 - Diagnosing Aneurysm of the Aorta

These diseases have long plagued humanity, and the challenges in finding them are not new either. The following words were written long ago by the renowned physician, William Osler, regarding diagnosis and aneurysm of the aorta (3), 

"The well-known dictum of Jenner may be taken as text: 'More mistakes are made by not looking than not knowing.' A majority of aneurism of the thoracic aorta present suggestive features to the eye, but the inspection must be made with care." 

Writing of having missed aortic diagnoses, Osler mentions an example of someone named McKinley. He continues with the often quoted:

"There is no disease more conducive to clinical humility than aneurism of the aorta."

Continuing, his words at the close of the page are compelling, where he quotes Pirogoff:

"Mistakes occur with the most careful and most skilful. Sometimes the diagnosis is beyond our art; more often it is not made because of the carelessness that so easily besets us in our work. The confession of the great Pirogoff always seems to me most touching: 'There are in everyone's practice moments in which his vision is holden, so that even an experienced man cannot see what is nevertheless perfectly clear, at least I have noticed this in my own case. An over weaning self-confidence and pre-conceived opinion, rarely a weariness, are the causes of these astonishing mistakes.'" 

It is over 100 years since Osler wrote about diagnosing diseases of the aorta and aneurysms, citing Jenner and Pirogoff (4).  The tools of modern technology have not solved the diagnosis challenges. The authors of these recent papers, several from Johns Hopkins where Osler came as one of the founders in 1888, are bringing much needed focus to the harms done when certain diseases are not found or not found quickly enough. May the transparency and humility of Osler, Jenner, and Pirogoff, coupled with courage in the face of deadly diseases, be inspirations today to overcome limitations and find mechanisms to reduce misdiagnosis.

The Challenge in 2021

Doug Grieshop's Diagnosis,
 BAV with Aortic Dissection/Rupture,
 Came too Late, During an Autopsy

Consistently diagnosing just a small number of diseases in time (aortic aneurysm and dissection, endocarditis, and sepsis among them), would drastically alter the ability of these diseases to disrupt and destroy lives, some of whom were born with bicuspid aortic valves. Someone with BAV has too often been discounted with "anxiety", sent home when a heart attack was ruled out. Some are disabled, some have died. 

The crisis eventually passes,

 but the scars linger.

May the new light these papers are shining

 be a springboard to improve diagnosis,

significantly reducing injury and death.

 ~Arlys Velebir,  Bicuspid Aortic Foundation

(1) Newman-Toker, David E., Schaffer, Adam C., Yu-Moe, C. Winnie, Nassery, Najlla, Saber Tehrani, Ali S., Clemens, Gwendolyn D., Wang, Zheyu, Zhu, Yuxin, Fanai, Mehdi and Siegal, Dana. "Serious misdiagnosis-related harms in malpractice claims: The “Big Three” – vascular events, infections, and cancers" Diagnosis, vol. 6, no. 3, 2019, pp. 227-240. https://doi.org/10.1515/dx-2019-0019

(2) Newman-Toker, David E., Schaffer, Adam C., Yu-Moe, C. Winnie, Nassery, Najlla, Saber Tehrani, Ali S., Clemens, Gwendolyn D., Wang, Zheyu, Zhu, Yuxin, Fanai, Mehdi and Siegal, Dana. "Serious misdiagnosis-related harms in malpractice claims: The “Big Three” – vascular events, infections, and cancers" Diagnosis, vol. 6, no. 3, 2019, pp. 227-240. https://doi.org/10.1515/dx-2019-0019

(3) Modern Medicine Its Theory and Practice, Volume IV, Diseases of the Circulatory System - Diseases of the Blood - Diseases of the Spleen, Thymus and Lymph-Glands, Chapter XI Aneurism,William Osler, MD, FRS, p472.

(4) The Life and Work of Nikolai Ivanovich Pirogov (1810-1881): An Outstanding Anatomist and Surgeon

*Bolding added to quotations through out.

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

"Bovine Arch" - Researching Another Aortic Mystery

Normal arch

More to the Aortic Mystery?
There are so many things not well understood about bicuspid aortic valve in individuals and families. Are there other aortic clues, beyond the abnormal aortic valve, that may be markers, important clues to aid in understanding each person? It is possible that one of those clues may be the aortic arch.

 "Bovine" Aortic Arch - Similar to Cows?
The aortic arch is the curve of the "candy cane".  There are three arteries that branch off from this curve delivering  blood to the head and upper body. Each of these arteries normally branch directly from the aorta, and each artery has its own separate opening, as pictured in the drawing on the right.

However, physicians have noticed that in some people their arch vessels are different. In medicine, the term "bovine arch" has been used to describe these differences.

Variation of the Arch Branches in People - Not Like Cows After All!
Bovine refers to cows, however, this is not an accurate description of what is seen in humans! The variation of arch branches in humans is not the same as that found in cows. Somehow, the name has persisted, although it is not accurate.

Here is a link to a paper that describes the "bovine arch" in more detail.

Bovine Aortic Arch Variant in Humans: Clarification of a Common Misnomer

For those who want to know whether or not they have a "bovine" aortic arch, it can be seen through CT and MRI scans, and may be described in the written reports of those tests. It is something which may be discussed with physicians in reviewing test results.

Please Support the Search for Answers
Those of us who volunteer with BAF know that the bovine arch is present in our families. We are very interested in understanding if this is also a useful marker, another malformation of the aorta that should be noted and understood more fully.

Some family members appear to have "normal" three-leaflet aortic valves, yet they develop aneurysms, "electrical" heart issues, and other issues in their bodies. They also have the bovine aortic arch. There is a great deal to explore further in the quest for answers and help.

The Bicuspid Aortic Foundation is supporting an effort to gather data about the arch variations present in those with bicuspid aortic valves. During these last days of 2018, looking forward to the new year to come, we invite you to join with us by supporting our search for answers. Your donations make this search for answers possible.

Online donations may be made through our MightyCause page.

Above all,

 we encourage everyone

to seek knowledge,

 to ask questions,

 and to never stop learning

 about bicuspid aortic valves and thoracic aortic disease. 

This is how lives are saved,

Creating a Climate of Hope,

~Arlys Velebir

                         Bicuspid Aortic Foundation

The Gift of Pain

Father Prodromos

Father Prodromos has had four surgeries, the most recent one a second repair of his aortic coarctation, which was first treated in childhood. The incision to access this part of the aorta is on the side, underneath the arm. He has lived with chronic pain since that surgery. Here are his reflections on his life now, written on this Christmas Day, December 25, 2016, from the monastery in Cyprus where he lives.

As I see it, one of human’s most common weaknesses is obliviousness. We tend to forget even the greatest gifts life has given us throughout our journey in time. So, it is no wonder that I honestly thank God for an unusual gift I was given by His all-caring and fatherly providence: pain!


Pain really is a mystery. Why some people find themselves in chronic pain after a surgery and some not? Why some people struggle all throughout their lifetime against this uninvited friend and some never even get the chance to meet him? The answer is never to be answered. One needs to find his own, very personal way to get along with the lifelong reality of pain. And for me, St Paul has portrayed the most blessed approach to this phenomenon. He writes:
“Therefore, in order to keep me from becoming conceited, I was given a thorn in my flesh… Three times I pleaded with the Lord to take it away from me. But he said to me, ‘My grace is sufficient for you, for my power is made perfect in weakness.’ Therefore, I will boast all the more gladly about my weaknesses, so that Christ’s power may rest on me. That is why, for Christ’s sake, I delight in weaknesses… in hardships… in difficulties. For when I am weak, then I am strong.”
Mrs Arlys Velebir was the one to correlate this verse by the Apostle to my state. She did that when at my first year’s anniversary after my surgery in 2015 I wrote to her the following about my pain: “every day we all face what our Lord allows to come in our lives, trying to make the best of it for the benefit of our eternal existence: perfect love to God and our neighbour under any circumstances. So, glory be to God for all things!”

Yes, I would forget about that wonderful miracle that took place in the operating room in January 2015. If you read my story on the BAF’s site, you will easily understand that what happened on that day was nothing usual. If I was in the hands of any other surgeon on that day it is really doubtful whether I would be now sitting and writing this. Not to mention that I would have never even made it to surgery. So, I had to remember; I have to always remember. But for this I needed this tiny little thorn. To others such a thorn might feel like a sharp blade. Not to me. To me is a wondrous reminder. It constantly reminds me that gratefulness is human’s most noble expression of love and understanding. I am so very grateful for this friend I was given, to walk along with him the remaining of my life and every single day to acquire the most precious knowledge from my relationship with him: “when I am weak, then I am strong!”

My dear friends, I have undergone 4 open-heart surgeries up until now with the possibility of even more to come. As the second anniversary of my last surgery is approaching, this is my message to all of you for new year: accept pain, every and any kind of pain -spiritual, sentimental, psychological or physical- with one thought: Pain makes us human. And only human can love. Therefore, the more we hurt, the more we love; and love is our destiny.

I trust that since this comes from someone who really knows what pain is, it should then count a little something to you! 


God’s love be with you all. Happy New Year!

Father Prodromos

Read the story of Father Prodromos' redo coarctation surgery experience here. 

September 2016 Awareness - Learning about Aortic Coarctation

What Did Sarah Have? Coarctation and BAV 
Sarah would be 20 years old now, a young woman. When I spoke to her she was just half that age and facing surgery. Sarah's Mom contacted the Bicuspid Aortic Foundation when they learned that Sarah was going to need surgery. A bright, athletic girl, Sarah began to have headaches. Doctors found she had high blood pressure, and further searching found both a bicuspid aortic valve and coarctation of her aorta. Sarah's Grandfather also had coarctation, they were not sure if had a BAV or not.

Here is a video from Children's Hospital of Cincinnati that explains what coarctation of the aorta is:

I spoke to Sarah's Mom first, and then she asked me if I would speak to Sarah too. What did I say to this beautiful, brave 10 year old girl before her surgery? I told her that she would be just fine, and that she was very special.  I also remember speaking to Sarah after she was out of the hospital. She did indeed do well, very seriously telling me that she was in the hospital for a very long time. If it is an eternity for adults, what must it seem to a child. They all so desperately want to go home.

I have not heard from this family for 10 years now, but I remember them fondly. I hope Sarah as an adult is experiencing all that life should hold for a bright, active young woman. I hope she has wonderful doctors and keeps her check up appointments faithfully, both for her aorta and her BAV.

Father Prodromos and Coarctation

More recently, Father Prodromos sought help from the Bicuspid Aortic Foundation. He was just a tiny tot when he had his first surgery, for coarctation of the aorta. In the intervening years, he has had three additional surgeries: BAV replacement, prosthetic valve and aneurysm replacement, and repeat surgery on the coarctation site.  Father Prodromos' last two surgery experiences are described at the links below.


Father Prodromos - My Journey of the Heart

Father Prodromos - My Journey of the Heart Continues

It is very common for those born with coarctation to also have BAV. May these accounts of those needing help first as children give hope to those today who must cope with this, no matter what their ages.

Sharing our lives

Together We Are

Creating a Climate of Hope.

All Best Wishes,

Arlys Velebir

Bicuspid Aortic Foundation

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.

"No Big Deal" - When Will BAV Be Respected?

This child did not require treatment
 until an adult, but that is not always
the case in those with BAV.

The email below was received
by the Bicuspid Aortic Foundation today.

After so many years of trying to raise awareness,
once again we must ask, how much longer
will it take for the bicuspid aortic valve 
to be taken seriously?

"My son was diagnosed with BAV when he was 10 months old, which was 5 years ago.  At that time they basically told us that it was no big deal, and he would just need to get checked every 5 years.  They told us that people go their whole lives not having any issues.

He just had his 5 year follow up Echo with a new cardiologist and his ascending aorta z score is 3.24, which we were told is moderately enlarged.  When the doctor told me this I just started crying.  How does this diagnosis go from no big deal to now his Aorta is enlarged?

The doctor really didn’t tell me much because he didn’t know how fast his aorta is growing since his last echo was 5 years ago.  He basically said to bring him back if he was complaining of chest pain.

We have so many questions and no answers.

I don’t know how concerned I should be, or if I need to restrict him from doing anything.  He is such an active kid, and it is impossible for him to stay still.

I don’t know what it means for his aorta to be enlarged like this at his age.  I have searched the web but I really can’t find a lot of information about children with BAV.  I am just trying to find out everything I can so I have a better understanding of how to help my son.  If there is any information you can give me I would greatly appreciate it."

Tears for Our Children, Our Loved Ones

I understand this Mother's tears. Those of us who volunteer with the Bicuspid Aortic Foundation have heard this too many times and have experienced it in our own families.

It is hard to take in adults, unbearable in a child, to go from "no big deal" to a bulging aorta with an unknown growth rate, to be told to watch for "chest pain"!

BAV May Be a Very "Big Deal" 

We all desperately want to believe that it truly will be "no big deal" in us. That we will go our whole lives without any issues. Yes, some people do.

No one can promise that to someone with BAV. 

Time reveals what BAV means in each person.

. 

What we need to know is what happens in some people with BAV:

• at some time in their lives, the BAV may leak, narrow, or both and need some kind of repair or replacement

• BAVers are at greater risk for infection of the heart and BAV 

• the aorta above the BAV may bulge (dilate) and over time become an aneurysm

We need to hear that we need lifelong follow up care, and that keeping copies of our records will help the doctors we see, who will care for us over many years. This way we give ourselves the best possible life, as knowledge and treatment get even better than today!

This Mom has excellent questions. The Foundation has tried to help her start learning about BAV. There are some physicians and centers that write about their experience with BAV children. We wish there were more who seek to better understand and care for children with BAV.

Here we share one such program for BAV children in Chicago that shares information with the public:   Children's Hospital of Chicago BAV Program .

Here is a page of medical information about children on the BAF website: BAV in Children .

And here are the stories of two little boys born with BAV: Emerson and Lincoln .

Our children

 deserve much more than

"it's no big deal",

much more than 

 being sent away for five years.

Together, we can share information

to help our children and 

their entire families.

Yes, together we are

Creating a Climate of Hope.

          - Arlys Velebir

                                     Bicuspid Aortic Foundation