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