Aortic Valve Disease, Blood Flow, and the Brain!

Aortic Valve Disease: Breaking through the Complexity

 

What is happening to me?

Perhaps there is nothing more frightening than knowing our brain is failing us.

Early this year, an intelligent, capable woman underwent extensive neurological testing. Along with some transient visual symptoms, she had increasing difficulty finding words. Stroke was ruled out. Several physicians suspected the onset of dementia. Only one neurologist said a definitive no, this is not dementia.

A few months later, in a city far from home, she could not breathe. She found herself in a very busy ER, where ultimately physicians and surgeons were challenged to save her. They did!

Today, as she recovers at home from an emergent, high risk surgery to replace her failing “TAVR in SAVR” prosthetic valve, her brain is clearly much better. 

While in retrospect there is tremendous gratitude for her “miracle” surgery and recovery, coming this close to the precipice, putting her life into the hands of physicians far from home in another city, is never by choice. Not for the person. Not for the physicians who confront the crisis. 

What could be learned from this? Were her brain symptoms an early indicator of what became an emergency originating with her aortic valve?

The following recent publication is very timely regarding the use of widely available ultrasound to aid understanding of blood flow, not just at the proximity of the aortic valve, but as it continues through the aorta to the body, and particularly up to the brain. Clicking on the title below opens the full paper.

Method of Recording Reverse and Delayed Turbulent Blood Flow in an Obese Pediatric Patient with Congenital Aortic Stenosis

I share here corresponding author John Leighton’s comment to the Bicuspid Aortic Foundation: 

“Any new loss of cognitive function in a valve patient whether a child or an adult is cause for concern.” 

At the Bicuspid Aortic Foundation we know, from our lived experience with aortic valve disease, that shared examples such as this paper’s description of one 12 year old, may often shed light on the plight of many others who need help too. 

The Bicuspid Aortic Foundation thanks the authors of this paper for their interest in and work on behalf of those with impaired heart valve function.

Piercing the mystery that clouds their lives,

shining light on their blood flow, 

Creating a Climate of Hope,

~Arlys Velebir

                     Bicuspid Aortic Foundation

Post Script

Why was surgery the only option, instead of a TAVR in TAVR in SAVR? Quite simply, the type of TAVR valve in her heart did not allow insertion of another TAVR without blocking the blood flow to her heart. Surgery was the only option for a new aortic valve. The Bicuspid Aortic Foundation is very grateful for those skilled hands that perform these complex surgeries, extending the lives of those born with bicuspid aortic valves.

Thoracic Aortic Disease Month September 2023 - Remembering Families

Aortic disease may be summed up in two words: "it hurts".

In words from the above, performed by Nazareth: it hurts, it scars, it wounds, it marks... 

Aortic disease also lies and deceives - it hides on the inside, it deceives, and then it "burns you when it's hot".

Aortic disease hurts whole families. It hides in various members of a family, some with bicuspid aortic valves, others in that same family with all three leaflets!

It is extremely painful to those who are alive when they begin to realize the potential hidden toll aortic disease may have taken on loved ones over multiple generations.

Bicuspid Aortic Valve and A Grandmother's Heart 

"We Thought That My Family Died of Heart Disease"

Especially remembering,

on this last day of September Aortic Disease Awareness 2023,

all the families hurt by thoracic aortic disease.

Thoracic Aortic Disease Awareness September 2023 - A Time to Remember

"Try to remember when life was so tender

That no one wept except the willow"

"Deep in December our hearts should remember

And follow, follow, follow" 

How long is 22 years?

I do remember a time of innocence in terms of aortic disease in the chest. It is 22 years now since a large aortic aneurysm was discovered around and above the mechanical aortic valve in my husband's heart. It changed our world. It became clear after his successful surgery that others needed to be warned, to receive help. How long has time been since then? Very long. And very short. Time is like that, its length defined by what happens in the moments of our lives.

Remembering our roots at the Bicuspid Aortic Foundation, this year we mark the passage of time since becoming conscious of the tremendous implications of this disease and the need for awareness and advocacy. Those issues and needs, along with unanswered questions, persist today despite progress made.

At the Bicuspid Aortic Foundation it is a privilege to once again focus on the aorta with September Awareness 2023. It is indeed gratifying to see many global efforts to raise awareness, support individuals and families, and continue progress through research and advancements in surgery.

This September we remember...

• We remember those who died.
• We remember those who survive with injuries.
• We remember all those with thoracic aortic disease, too often undiagnosed.
• We remember the great pioneering medical knowledge and surgical progress that saves lives through out the world today.

At the Bicuspid Aortic Foundation we are
Remembering this September;
And along with our memories,
Creating a Climate of Hope,
~Arlys Velebir
                                          President, Bicuspid Aortic Foundation

September Awareness 2022 - Breaking the Pattern of Sudden Death

This September 2022, we reflect on the 20 year experience of one man. Despite the odds against him, he is not only still alive but fully enjoying life in his early 70's! While there is special focus on the aorta in September, his experience is a reminder that there can be multiple dangers, both heart and aorta, to someone's health and life. 

Family History - 14 Men, Sudden Death Before Age 65

Willie, not his real name, has a formidable history of sudden death in his father's family. Fourteen men "dropped dead" before the age of 65. They never reached the hospital alive.

What was it about these men that doomed them? What took them suddenly? Was it a massive heart attack? Could it have been something very different, a torn or ruptured aorta in the chest? What about sudden death from undiagnosed aortic valve disease? Willie has managed to proactively prevent harm from all of those things: aortic valve, aortic aneurysm, coronary artery blockage.

It could have been very different, as it was for many in his family.

Willie was born with BAV, but like many he had no awareness of it. Discovered at around age 50, his BAV was not working well and his aorta was bulging above his heart and needed surgery. Fast forward 20 years after successful aortic valve and aneurysm surgery, and his biological aortic valve was in major trouble.  He also had developed CAD (coronary artery disease)! 

All of these reached a point where he needed treatment, but all of them were also "silent" in his body. No symptoms. When asked how he feels now, after the most recent treatments, Willie says he feels well but adds that he has never felt unwell. No chest pain, no shortness of breath, nothing....

Today, he is a triumphant example of living well despite these silent threats, due to:

• proactive diagnosis 
• seeking information and second opinions 
• timely treatment
• ongoing care 

Breaking the Pattern of Sudden Death

Willie has overcome that horrendous history, and is grateful to be alive and well now, as he enters his early 70's! Even without any physical warning signs,  he was treated in time to prevent injury or sudden death from:

• bicuspid aortic valvular disease
• ascending aortic aneurysm
• prosthetic aortic valve failure
• coronary artery blockage

It is very special to share in his joy in being alive and well.

During September Awareness 2022,

 we share his proactive triumph

over BAV/TAD

and CAD,

Creating a Climate of Hope, 

                    ~ The Bicuspid Aortic Foundation

September Awareness 2022 - Larry Abramson 1955 - 2021

Larry and his wife in 2008,
after aneurysm/valve surgery

In September 2019, Larry gave the following to the Bicuspid Aortic Foundation, to encourage others and raise awarness about the aorta in the chest. It is painful to realize that just two years later, on September 22, 2021, he did not survive a 4th open heart surgery, a fight forced by infection.

September Awareness 2019 - Larry Abramson 

I remember with sadness the last time I heard Larry's voice, no longer strong and clear, the tone roughened by his illness.

 Larry was very unwell by then, after days and weeks of ups and downs - testing, searching for answers, reaching dead ends - had become months.

At last, the villain was cultured in his blood, the infection’s damage to his heart became clear, and one more surgery, his 4th, was his only hope.

Larry did not survive that last surgery. The infection’s damage was too severe.

Devastating bacteria have evolved, with the ability to conceal their presence and aggressively attack the heart and its valves until extensive damage is done. Such an infection ended the life of this amazing man, born with BAV. 

It was in the "early days" of heart surgery that he had his first, at age 11. From his second surgery at age 20 onward, he lived with mechanical aortic valves, a span of 44 years. This account by his family speaks to the vibrant, active man he was, until those last weeks. 

One year later, 

the Bicuspid Aortic Foundation remembers Larry, 

who lived so vibrantly with "great heart",

 and was taken much too soon.

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

BAV in 2022 - We've Come a Long Way, Still a Long Way to Go....

Heart Month 2022 

Sacagawea - Arduous Journey 
Beautifully portrayed here
along the shores of the Missouri,
 she inspires my own journey! 
We can do this!

It is not surprising that a recent mailing from a local hospital features an article about the heart. What is wonderfully amazing to me is that this is not an article about coronary artery disease, it is a BAV aneurysm surgery story - a hopeful account of a man and his family receiving care locally. I am joyfully amazed! 

We have indeed come a long way since 1990, when my late husband had his first surgery in that same hospital, where his BAV was replaced. 

Along with the article is a picture of a healthy, strong man and his wife, a variation of myself and my late husband. He is about the same age as my husband was when his BAV took center stage, demanding attention!

Highlights of their story:

• Diagnosed with BAV about eight years earlier, his cardiologist had "followed" him since 2012
• On July 4th, 2021, while on vacation, without warning his heart began to race; paramedics were called, he was experiencing SVT, and received local care
• Returning home, further testing revealed severe aortic stenosis, and he met his surgeon
• His BAV and ascending aorta were replaced in September 2021
• After surgery, he also needed a pacemaker, which was implanted before he went home
• They were told that this is genetic, and his children underwent screening 
• He is back to his work, interests, and looking forward to traveling; the article quotes him as saying he feels great 

We've Come A Long Way

In 1990, valve and bypass heart surgeries were being done in this relatively small hospital, which has grown significantly since then. Our experience:

• Unusual for him, my husband was very sick, abruptly, expressed by pneumonia and heart failure
• The pneumonia thankfully was treated successfully
• Despite a heart murmur from childhood, after testing we heard the words "bicuspid aortic valve" for the first time
• Nothing was said to us about his aorta, although his pre-surgery catheterization report documents "post stenotic dilatation" of his aorta; dimensions of his aorta not noted
• His BAV stenosis was "critical", his left ventricle had thickened and distorted ("boot shaped")
• Adding to the drama, the local paper ran stories about a certain mechanical valve breaking inside people's hearts 
• His surgeon safely replaced his extremely calcified BAV without particles escaping and causing a stroke 
• Surgery gave him a mechanical valve and his life
• We were told he was "fixed for life", with no restrictions or changes other than cautions about warfarin interactions, keeping his blood coagulation within range (measured in seconds, later by INR), and warnings about the risk of infection (endocarditis); he resumed his favorite exercise regimen, lifting heavy weights
• Generally, he was not viewed as unusual in any way because he had a BAV; BAV persons were treated the same as any other aortic valve requiring surgery at that time. His blood was carefully followed, the valve less so, and the aorta not at all.

When eleven years later, a large aneurysm above that mechanical aortic valve was fortuitously discovered, icy fear gripped me. I knew at that time if that aneurysm caused a crisis, the local hospital could not handle it, even if he made it there....  It was not easy, but we informed ourselves (online), and made a list of major centers performing ascending aortic surgery in the United States. Mercifully, the integrity of his aorta was not breached and highly successful aortic surgery gave him his life, again.

Yes, It Can Be an Arduous Journey

Some have more complications than others. Only with time will each person's journey unfold. They need individualized care. For many, it is both an individual and a family journey. We celebrate every advancement, every victory, but must remain vigilant against the unknown, the unplanned, the dangers....

Still a Long Way to Go

There are many frontiers to explore regarding BAV persons and their trileaflet family members. I hope many drawn to the heart early in the careers will be inspired to press forward deeper into these frontiers. This is a large population with many that need you.

Focusing here just on BAV and aortic stenosis, understanding when they need intervention can be challenging. "Following" BAV people is not trivial. This man, like my late husband, abruptly "got into trouble" with his BAV stenosis. This has been a trend in my family, and others I know well. Mechanisms for understanding them better, especially when they do not express any significant symptoms/warning signs to alert anyone, is an area ripe with opportunity. His cardiologist remarked at the time on how well my husband's heart muscle recovered after that first aortic valve surgery. Not everyone is so fortunate.

Better indicators for those with BAV would facilitate well-timed, proactive aortic valve interventions - not too soon and not too late - enabling them to fully achieve their potential. So many with BAV are  "youthful" regardless of their age, active and gifted in so many ways.

Along with optimal timing of aortic valve intervention, there is choosing the optimal aortic valve solution for each one. The article does not mention which type of valve replaced this man's BAV. I remember the first time I heard it said "There are no perfect valves" for replacement! Importantly, this man's prosthetic aortic valve, pacemaker, and repaired aorta position him for life - life with ongoing, lifelong care following this first surgery. Reflecting from my perspective now, with the passing of time, I know just how young this man truly is.  For him and his family, and the many, many others just like them, where ever they may be in the world,

May your future journey be safe,

smooth, and long!

When there are bumps in the road,

always remember

we can do this!

Making progress together,

Creating a Climate of Hope,

~Arlys Velebir, Bicuspid Aortic Foundation

When Sunset Comes too Soon - Remembering a Woman with BAV

Pacific Sunset 

This is written in remembrance of Julie . . . a wife, mother, grandmother, and so much more... I am calling her Julie here, not her real name, to preserve her privacy and that of her family.

She passed away suddenly, unexpectedly, about a month ago, on a day in early January 2022, not very long after saying goodbye to her own fragile, elderly mother in December.

She had no inkling that her own life would not be such a long one, that it would end suddenly that January day. Seemingly neither did anyone else. 

Her husband of over forty years was right there with her when her heart stopped, never to beat again despite all efforts. His love for her and their family enabled him, despite the tremendous shock and grief, to obtain the best private autopsy available. 

Among the findings listed, the pathologist examining her heart found her severely calcified stenotic bicuspid aortic valve and the enlarged left ventricle that had worked so hard to push her blood out to her body.

No one, not Julie, not any of the physicians she had ever seen, knew this about her.

There is one mention in her medical records, in 2018, of a heart murmur - seemingly heard just that once. No echocardiogram was done.

Other than being a "little tired" sometimes, so easily dismissed, there were no symptoms that might have prompted further investigation of her heart.

The autopsy report includes advice to her family to be screened since other blood relatives may be affected - a lifesaving gift to this family from their dearly beloved mother and courageous father, who must carry on without his soulmate. Along with Julie's BAV AS (aortic stenosis), all of the findings the autopsy reveals will help her family understand as much as possible about what caused her sudden death that day. 

It is not the first time I have spoken with someone shocked and grief-stricken by the sudden death of their loved one, where the bicuspid aortic valve, not the aorta is implicated.  Sometimes, as in Chuck Doherty's Sudden Death, Age 34, the presence of a bicuspid aortic valve was known to them and their physicians, but for others like Julie and her family, it remained unknown until after death.

May these deaths inspire us all to continue pressing for diagnosis in the living and greater understanding of those with BAV.

This Heart Month 2022,

 we remember Julie,

 a very special woman,

born with a bicuspid aortic valve, 

whose sun has set too soon. 

~Arlys Velebir,  Bicuspid Aortic Foundation

 

Christmas 2021, a New Year and "Lucky to Be Alive" , January 2022

Heart Friends for Over 20 Years 

Red Jasper,
for Heart Warriors
 Everywhere!

I have known this man for something over 20 years. After my late husband's aneurysm diagnosis, he is the first person I met who had a bicuspid aortic valve and ascending aortic aneurysm. He had been told that he had both of these in his chest, and he was struggling to find expertise and help. Compared to today, 2001 was the "dark ages" in terms of even basic information about this combination, and he responded to my very first attempt to share information online.

Merry Christmas 2021

He had his surgery, and we have stayed in touch over the years since, often at Christmas. This year, in my response to his Christmas Day text greeting, I included a link to a TAVR expert whose skilled hands have saved a challenging member of my family as well as others that I know well, with valve-in-valve as well as native BAV TAVR procedures. 

My friend's surgical valve was rather ancient after 20 years, after all! His bovine pericardial aortic valve had defied the typical expectations for someone well under 60 at the time it was surgically implanted! Amazing!

January 14th, 2022

When I saw his name, I was delighted to answer the phone! I thought perhaps he was calling to chat about where he was in terms of his valve and researching his options.  

His first words shocked me, but in a good way! He was in a hospital bed - "I had TAVR this morning"!

Fast Response, Expert Hands, Success!

In the brief time since Christmas, he had contacted the TAVR expert I mentioned, sent his records, and traveled from out of state to be seen. Based on what he was hearing from physicians at the major center where he lived, he thought he might be told that he would need something done in a few months....

After having state-of-the art imaging, he was in for a major surprise! Yes, his bovine valve needed replacing, but not in weeks or months. His valve was urgent! He would have TAVR in two days! 

More to the Story

• Like many I know, he did not have symptoms, at least that he recognized, that would tell him he was in trouble - that his old bovine aortic valve was badly leaking! 
• In addition, he was told that he has a significant, dangerous blockage of one of his coronary arteries. He will be returning soon to have that addressed. He had never been told this! Yes, some born with bicuspid aortic valves also develop "the other" heart disease, and he is one of them....
• He had kept detailed records of his heart history, over all these years, in a spreadsheet, and provided that when seeking this opinion. Moving from one state to another, seeing various physicians over the years - this was very important!
• Things that were said to him locally made him uncomfortable, and moved him to seek expertise elsewhere.
• He was able, financially and physically, to travel from another state. 

What Next?

He ended a recent message to me this way:

"What next? Lucky to be alive!!"

May his coronary artery stay open until his upcoming procedure, and may he have many more good years, made possible by very timely discovery and expert treatment of his beautiful heart (and aorta) - twenty years ago, and once again, now!

In 2022, may we continue

 to pursue our best options,

 Creating a Climate of Hope,

~ Arlys Velebir

Bicuspid Aortic Foundation

September Awareness 2021 - TAD and the Tip of the Iceberg

 

The Tip of the Thoracic Aortic Disease Iceberg?
Photo by Danting Zhu on Unsplash

At BAF we have spent the month of September 2021 focusing on those who need life-long thoracic aortic care. 

It is so encouraging to see the increased global awareness of thoracic aortic disease. It has come a long way in the 20 years since my personal experience with my husband began. In the closing hours of this month, I am caused to reflect with gratitude on that which is seen and known - that which is above the water, so to speak.

Below the Water Line

However, those unknown, unseen, "below the water line", are those with unmet TAD challenges, those not receiving life-long aortic care. Who are some of these people? 

• the undiagnosed
• diagnosed with bicuspid aortic valve (BAV), aorta not evaluated and monitored
• replaced BAV, prosthetic valve monitored but not aorta
• TAV relative of someone with BAV, aorta not baselined or monitored
• prior elective aortic procedure, such as replacement of a portion of the ascending aorta, remaining aorta not monitored
• prior elective aortic surgery for coarctation, not receiving effective ongoing monitoring

What about those who survive aortic dissection?

Are those who survive an aortic dissection ever presumed to be "fixed"? Do they become "lost to follow up"? Those we have met at BAF are receiving careful monitoring, perhaps because of their physicians' appreciation for their very survival and the injury done to their aorta. If any of them are not receiving ongoing monitoring and additional intervention as needed, they are also at increased risk, below the water line.

The BAV/TAD Population - 6.7 Million People in the US Alone

The BAV population is large: estimated at up to 1 in 50 people (2 %). Checking the census bureau population clock, it displayed the number of people in the United States as 332,800,350 people. Two per cent, or 1 in 50, is 6,656,007 people born with BAV in the United States alone. 

Whenever I am in a group of 50 people or more, I wonder about those around me, more frequently they are males, born with a two-leaflet aortic valve in their heart - possibly already replaced. I have met some of them over the years, sometimes in such unexpected settings.

Some statistics mention that 5% of those with BAV dissect. Five percent sounds small. In the financial world, it's only 5 cents out of a US dollar. When applied to a large number however, such as 6.7 million, that relatively small percentage yields a six-figure result: 335,000.  

When applied to humanity, think of the tremendous number of BAV lives with a torn aorta in the United States alone.  However, this is a global, not just a United States, problem. 

The BAV/TAD View in 2021 

Twenty years ago, it was very difficult to find information and help for an "ascending aortic aneurysm", but we did it.  Knowing how challenging it was, we were moved to help others, many who also no doubt thought they were "fixed for life" by a BAV replacement.

There are still those today with a replaced BAV and presumably receiving good ongoing care, who have no idea their aorta is bulging, endangering them. I spoke to one of them quite recently, whose BAV was replaced 9 years ago. This person is above the TAD water line now, the bulging aorta exposed, receiving comprehensive aortic care.

I ponder once more how many with BAV/TAAD die undiagnosed, uncounted, a "natural" death without autopsy; perhaps an in-hospital death due to aortic dissection, but the aortic valve condition unspecified. How large is the iceberg beneath the water?

In 2021, we are in a better place in many ways, but not yet where we aspire to be in terms of BAV/TAD. We look forward to new advancements that will come through the efforts of gifted physicians, surgeons, and researchers around the world. 

As those affected 

are included

 in the heart/aorta teams of today,

 together we will advance,

 lowering the water line,

more receiving life-long aortic care,

Creating a climate of hope,

~Arlys Velebir, Bicuspid Aortic Foundation

 

September Awareness - Lifelong Aortic Care after Elective Surgery

Diseased Thoracic Aorta

One Man and his Aorta - Years after Surgery, Another Aneurysm!

This September I am remembering one particular man and his wife. Although I only met them once, well over a decade ago, their experience remains with me. I share it this September, in terms of the aorta and life-long aortic care.

He appeared to be a strong man, in the prime of his life, who had not been given any specific "label" for his TAD. His wife was with him, deeply concerned about her husband, as together they sought more information. He had already had a voluntary aortic surgery years before, when quite young. His aorta had never torn (dissected).

Aortic Arch Aneurysm - "Maybe it has always been this way!"  

 I recall his wife saying those words -  hoping against hope that this recent picture of his bulging aortic arch was just the way his aorta had always been - that nothing was actually wrong. TAD, silently progressing over years, makes it far too easy to spend time in denial. 

This reference describes isolated aortic arch aneurysm as "uncommon": Aortic Arch Aneurysm . 

Uncommon, perhaps, but it had happened to him. He had perhaps a little knowledge of what his aorta was like right after it was repaired. However he had not had "aortic pictures" taken from time to time since then - pictures that could have captured the changes in his aortic arch that now had become this pronounced bulge. Whatever had prompted the recent imaging, these pictures very clearly had captured the aneurysm of his aortic arch.

In time, as they continued their search, they may have come to realize that, however unwelcome the discovery, it gave them the opportunity to avoid an aortic crisis - time for opinions from surgeons who would assess his aorta, time for them to ultimately choose an aortic surgeon to skillfully repair his arch while protecting his brain. I hope so.

Ongoing Monitoring, Baseline After Surgery, Lifelong Aortic Care

Reflecting on the aortic care my family has received over the past 20 years, I am thankful. Once an aortic enlargement or aneurysm in the chest was found, there has been ongoing monitoring, taking those "aortic pictures". 

The importance of blood pressure monitoring at home and medication as appropriate has been emphasized.

After surgery, we understood the extent of aortic repair, and a baseline image of the repaired aorta was captured for future reference. Then, back to appropriate, periodic imaging.

This is the essence of the Aortic ABC's

We do not outgrow the Aortic ABC's after Surgery.

 Surgery sometimes includes both aorta and BAV;
and then, back to life-long follow up care.

The Bicuspid Aortic Foundation defines the "Aortic ABC's" as follows:

• Accurate diagnostic testing
• Blood pressure management
• Comprehensive, individualized Care

There is great concern for those who do not  receive ongoing aortic care after a planned, successful surgery - perhaps stemming from the thought that aortic surgery is a one-time, life-long "fix".

Elective surgery may have repaired the aorta. How long will that repair last? Will some other portion of the aorta, years later, require attention?

 For a long life,

 as long as we live: 

Life-long Aortic Care,

Creating a Climate of Hope,

~ Arlys Velebir, Bicuspid Aortic Foundation

September TAD Awareness - Life-long Aortic Care for Coarctation

The Right Emergency Care at the Right Time
An account published earlier this year describes what happened in 2018 along a stretch of Interstate 90, as one man returned home from vacation with his family. I share the article here:

Heart emergency - right care at right time

Twenty four years after his last aortic surgery, now age 44,  an aortic aneurysm was dangerously bulging in his chest. Who can say why, at that precise moment, his abnormal aortic tissue could no longer hold together and contain the blood pumped with each heart beat? 

In the article, Dr. Jason Knutson describes many things that went right, one after another, in saving his life. He mentions not knowing that the odds of survival were about 2% in a hospital, less out on a highway!  

Yes, he received the right emergency assessment and surgery at just the right time. Reading this article again now, for September Awareness 2021, my initial question remains: why didn't this man have ongoing aortic care and a scheduled, elective surgery to address his aortic aneurysm? 

In the article, Dr. Knutson mentions that after that first surgery, thinking everything was ok while growing up, an athletic heart screening revealed that another surgery was needed; the patch placed on his aorta at age 6 had not grown with him. He had surgery again at age 20. 

Father Prodomos' coarctation
"redo" surgery, patch
and rupture visible

Patching of coarctation was also the initial surgical relief that Father Prodromos Nikolaou had at age 2 in 1981, not repaired again until over thirty years later in 2015 as he writes here: The Hidden Danger: A Patched Coarctation . How long had it seriously endangered him before being addressed?  As pictured, surgery removed that section of aorta completely. It is important to warn others of such life-long risks!

Life-long Aortic Care - "the right care at the right time"

For those wishing more detail, this full paper from 2015 covers coarctation, including BAV and aortic aneurysms also.

Current management of coarctation of the aorta 

Regarding surgical solutions, the paper states that the patch solution is "avoided whenever possible due to the frequent occurrence of aortic aneurysm and rupture".

Prior to their conclusion, the authors write of this as a "lifelong disease" and the importance of "life-long surveillance", whether the coarctation has been repaired or not. 

The knowledge exists.  Are people receiving the care?

Life-Long Aortic Care and Coarctation

In the early years of  BAF, we came to know a talented young athlete who was diagnosed with BAV and coarctation. He had surgery while still in his teens. His family became well informed and understood the importance of ongoing vigilance.

We were also contacted by a very worried Mom. She had taken her 10 year old daughter to the doctor because of headaches. I will call this little girl Sally. The bottom line, following testing: Sally's aorta was narrowed, just past the arch (coarctation), restricting blood flow and building up pressure in the vessels feeding her head.

After I spoke with her Mom, she put Sally on the phone. I still hear her sweet voice across the years. I spoke to her once more, after she was out of the hospital. She told me in a very serious tone that she had been in the hospital "a long time" - not so long really, but it is always too long, even for adults - how much more at age 10!

Her Mom shared Sally's picture with us at BAF. A lovely young girl then, Sally would be in her 20's now. I hope she is living the active, vibrant life that awaited her then. I would be so happy to hear all about her life now. Most of all, I want to know: is Sally receiving Life-Long Aortic Care? 

For those with coarctation,

Life-Long Aortic Care

is

 "the right care at the right time"

~Arlys Velebir, Bicuspid Aortic Foundation

September Awareness 2021 - Why "TAD" Awareness

2001  and "TAA" (Thoracic Aortic Aneurysm)

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

2005 and Thoracic Aortic Aneurysm and Dissection (TAAD) Awareness

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

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

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

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

2021 and Thoracic Aortic Disease (TAD) Awareness

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

One Man's Life-Long Aortic Journey Until Now 

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

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

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

Journey Beginning  from Age 2 

Hidden Danger of Patched Coarctation

"So, life is precious"

 as Father Prodromos wrote,

and life can be extended 

for those with TAD

when they receive

 Life-Long Aortic Care.

~Arlys Velebir, Bicuspid Aortic Foundation

September 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.

BAV Lifelong Care - Carrie Mettler's Journey - 2016 to 2021

May 2021 - Life is good!

Background - Carrie's BAV Discovered in Early 1990's

Carrie was born with BAV, but she did not know this until she volunteered to be a guinea pig for a new echocardiogram machine in the early 1990's. There it was - a two-leaflet aortic valve! The next year, after her sister's diagnosis included not just BAV but an ascending aortic aneurysm, Carrie had another echo which showed that her ascending aorta was enlarged also.  

Carrie already had begun her heart journey with the implantation of her first pacemaker in 1989, when she was only 29 years old. In the following years, her pacemaker journey alone has required multiple interventions due to infection, broken pacing leads, and device upgrades. In 2014, open heart surgery was required to address complications caused by pacemaker leads! Carrie had developed Superior Vena Cava (SVC) syndrome, which is obstruction of the blood flow though the superior vena cava. The cause was old pacemaker leads located there and scarring that had developed in this blood vessel over time. During surgery, Carrie's own pericardial tissue was used to enlarge her right atrium and patch her superior vena cava, and the abandoned pacemaker leads were removed.

Here, we focus especially on the most recent five years of Carrie's journey, from 2016 to the present. Carrie began experiencing chest pain/discomfort that year, causing her to seek out the expertise of the Cleveland Clinic. The remainder of this post are are in Carrie's voice, with paragraph captions added.

October 28, 2016 - Chest Pain Prompts a Visit to Cleveland

So off I went for evaluation at Cleveland Clinic.  I flew to Chicago where my little sister, Theresa, picked me up.  We then took an all-day road trip to Ohio.  We spent several days there.  I had an echocardiogram, a CT, labs and EKG.  Once all the tests were completed we met with a cardiologist as well as Dr. Eric Roselli, a cardiothoracic surgeon.  Dr. Roselli viewed the CT result with us, where we were able to view the actual scan.  My aorta was stable and they all felt that it was reasonable for me to continue on with the watch and wait approach.  The chest pain issue was still unsolved so I just had to back off on my running and switch to walking.

 So, over the next several years I kept up to date with my local cardiologist, where serial imaging of my aorta and bicuspid aortic valve remained relatively stable.  Chest discomfort remains unsolved.

March 2019 - Diving Competition, Bee Sting and  Breast Cancer

During a dock diving competition where my little dachshund was competing I was stung by a bee on my left wrist.  Initially the sting was painful but I continued on with the day just feeling tired.  Then I developed a large localized reaction which spread up past my elbow.  After seeing my primary care provider he instructed me that if the redness continued going up my arm that I should go to the ER.  I did end up going to the Emergency Room where I was given appropriate medications and steroids.  The “cellulitis” finally resolved.  It was also deemed that I am allergic to bee venom.

Bee sting reaction!

But something wasn’t right.  I just kept feeling like I had an ache in my left armpit area for a while…then I started feeling pain in my left breast. 

I was watching a nightly national news program one evening and they aired a segment about women with fibrous breast tissue should be screened with 3D mammography.  After seeing this and knowing my mother passed away after a battle with metastatic triple negative breast cancer…I called my PCP and asked if he would order this mammogram for me. 

 I went in for the mammogram the end of April.  The next day my doctor’s office called to tell me that I had a suspicious lesion in my left breast and that I will be scheduled for an ultrasound.   After the ultrasound my PCP called me to let me know I needed to have a biopsy. 

The biopsy was done on May 2^nd.  Four days later, I got the call…You have cancer!  My knees buckled and I felt sick and so frightened.  I got my wits together and went out to where my husband was working in our yard.  It was all so surreal.  Right up there with when I found out I had a brain aneurysm.  All of the results came back, and I indeed had the same cancer that my mother had.  Triple negative breast cancer.

Cancer Surgery and Chemo

There were many appointments and consultations, then finally I had surgery to remove both breasts June 24, 2019.  I spent a few days in the hospital and had the support of my husband, Vic, my sister Theresa and several of my dearest friends.  I actually felt pretty well and was optimistic as no cancer was found in my lymph nodes and the tumor wasn’t too large.  But it was still considered a high grade tumor and I had to be scheduled for 16 weeks of chemotherapy.   Prior to this I had to have a pre-chemo echo…where everything appeared stable.

After discussing everything with my oncologist and surgeon they agreed that I could take the RV trip we planned to our favorite park on the Metolius River in Oregon.  I was very grateful for this time to reflect and be with my husband and my sister by heart, Judi and her husband Greg.  We were also joined by two more dear friends one of which was a retired nurse.  I was in good hands and I felt the trip to be restorative.  

January 2020 - Last chemo treatment

August 5^th 2019     I received my first round of chemo.  Judi was with me for every infusion from then on.  I developed most of the nasty side effects.  Anemia, neutropenia, oral thrush, nausea and hair loss.  We had to postpone several treatments until my labs rebounded.  I also had to have several blood transfusions. 

I was pretty much exhausted most of the time but pushed myself to walk my dogs as much as possible.    In October I had another echo, and my cardiologist told my aortic valve was now mildly stenotic.  Fast forward to December and the aortic valve showed moderate stenosis.  I became more fatigued and short of breath and even after chemo ended in January of 2020 I still could not regain my stamina.  My cardiologist recommend that I work out but I could barely climb a flight of stairs.

 March 2020 - COVID, Fatigue, BAV Severe Stenosis, A New Door Opens!

The Covid 19 Pandemic lock down started.  I was supposed to have the tissue expanders removed and proceed with breast reconstructive surgery.  This procedure was deemed elective and my case was cancelled indefinitely.  I still felt short of breath, and fatigued along with exertional chest discomfort.  Not to mention how uncomfortable the tissue expanders were. Then in May my cardiologist ordered another echo.  My aortic valve stenosis was now severe.  I had mild swelling of my ankles and was becoming depressed and very frustrated. 

One day in August, my husband and I were driving down our road and stopped in to see our relatives that were out in their yard.  This was one of those divine intervention moments.   You see, this family member also has severe aortic stenosis!  Two of us, living on the same road, with the same problem. We talked for a while, and she told me that she had become discouraged with her local cardiologists and finally went to UC Davis in Sacramento, where she felt she receives superior attention.  I told her that I was also frustrated with my care and that my cardiologist of many years was set to retire.  I asked her to get me some contact information the next time she went.  A few days later she stopped by with the TAVR (Transcatheter Aortic Valve Replacement) Nurse coordinator’s card and said “she knows you”!  I looked at the name on the card and couldn’t believe that it was someone I used to work with in a cardiology office where I worked for many years.  She actually helped train me in pacemaker and ICD follow up….and we even had golfed together!  She was expecting my call!  I felt so relieved and thankful! 

 September 1^st 2020 - BAV Needs Replacing, Stable Aorta

Carrie's aorta 
2016 at Cleveland Clinic

Judi picked me up and off we went to UC Davis, where I had another echo and was evaluated by the cardiologist specializing in TAVR. I was also evaluated by a cardiothoracic surgeon and many medical students.

They all agreed that my aortic valve needed to be replaced, but they needed more time to go over the echocardiogram results and the recent CT scan.  Because I had previous open heart surgery for SVC syndrome and also have an ascending aortic aneurysm, I was considered high risk.

I felt very comfortable and confident in my new care team, knowing they would take time to make a treatment plan. Two days later I received a call from the TAVR nurse.  They determined that I was a candidate for TAVR and that my aorta was considered stable. 

 October 5, 2020 - TAVR in Native BAV

I had a transcatheter aortic valve replacement (TAVR) using an Edwards Lifesciences Bovine valve.  Everything went very well and I felt the difference immediately!  My physicians are optimistic that with my new valve, some of the pressure will be taken off of my ascending aorta.  We will continue to monitor my prosthetic valve along with my aneurysm. 

June 2021 - Blessed in the Face of Adversity

Today, I feel very well!  I was finally able to have my breast reconstructive surgery… also at UC Davis.  In fact, I have transferred all of my care to UCD.  My care has been amazing!  Yes the past few years have been a challenge…as they have for all…but again, I would not change a thing.

Feeling blessed in the face of adversity is truly a gift…and I will always be thankful for the bee and the cow that helped save my life! 

Carrie has been with us at BAF since our beginning days,

 and it is our privilege to walk with her always. 

Thank you, Carrie,

 for once again sharing your courage and strength with the world,

and through each experience,

 Creating a Climate of Hope

~Arlys Velebir

                    Bicuspid Aortic Foundation

Also see Carrie Mettler - A Wonderful Athlete with BAV   and 

Carrie in 2016 - Seeking Opinions for a Complex Situation