The Third Man

Thirty Years

As 2020 comes to a close, today I reflect on the experience of three men who each had aortic valve replacement surgery from a thirty year perspective.

What was it like for people who needed aortic valve replacement in 1990? 

Full open heart surgery was required to replace the aortic valve. Surgical replacement with an artificial heart valve had about a thirty year history then, dating back to roughly 1960. 

What kind of artificial valve would a given person receive? It seemed straight forward at that time. There were two general kinds of artificial heart valves: mechanical and tissue.

For younger people, with the exception of women who wished to become pregnant, a mechanical valve would be used, requiring anticoagulation (warfarin) to prevent blood clots and strokes. Taking warfarin required periodic blood tests. Warfarin patients were also given dietary restriction counseling.

Older people were offered tissue valves projected to last about 10 years, allowing them to avoid living with warfarin. 

Thirty Years Later, 2020

These three men all received mechanical aortic valves, valves that were intended to be a lifelong, durable solution for each of them. After healing from surgery, all three men resumed their lives.

Two of these men continue their lives with those original mechanical aortic valves. They have reached "old age" now and have developed other health problems. However, their original mechanical valves continue, just as intended, to open and close with each beat of their hearts.

The third man's experience turned out to be very different. 

The Third Man

The third man lived for 27 years following his first surgery. Between 1990 and 2017, he underwent a total of four open heart surgeries and experienced a major stroke due to valvular strands and pannus that had formed on that original mechanical aortic valve.

He lost his life following a tremendous fight with sepsis and persistent endocarditis due to resistant bacteria introduced into his blood stream at the time of the fourth open heart (third valve) surgery.

Was there something different about this man, the third man? Yes, he was born with a bicuspid aortic valve (BAV). 

How do I know so much about him? When I  met and married a man with a heart murmur, the future unknown to us then as it is for us all, I married the third man.

Much of what I have learned about BAV and thoracic aortic disease (TAD) is from what became our relentless pursuit of answers and help, as we faced together yet another battle, too often an ambush, when various complications confronted us. Far from being "fixed for life" as we happily believed when told this in 1990, we would become, out of necessity, readers of the medical literature, keepers of medical records, and searchers for answers along with the physicians and surgeon who bravely helped us.

We remained hopeful that each intervention would be the last, but over time our "heart journey" was much more like a convoluted roller coaster than the hoped for predictable path forward. 

My beloved husband's life ended that way, after months of precipitous ups and downs, with so many unanswered questions about the mysteries, the most obvious clue being that two-leaflet aortic valve replaced in 1990 and the large ascending aortic aneurysm "discovered" in 2001. There were other congenital "anomalies", somehow part of the package accompanying being born with a bicuspid aortic valve in this man. Eventually, I have recognized some of those things in others who also have been born with BAV.

2020: What has Changed?

It is three years now since this one man's last battle ended, but the fight continues for others living with BAV/TAD and their physicians. Many questions, that persist since at least the days of Abbott and Osler, remain today for pursuit by those in research.

Some born with BAV, like my husband, will take their physicians far beyond what is known, out on the frontier so to speak, where unexpected battles are fought and only sometimes won.

At this time, the big change regarding aortic valve disease is the availability of TAVR, which I first learned about in the 2005/2006 timeframe. My family and others I know have clearly benefited from this technological innovation. However, along with this new option come numerous questions to be carefully considered for those with aortic valve disease, perhaps most especially younger people born with BAV. 

What Will Another Thirty Years Bring?

In the context of 60 years of history, what does the future hold? 

In our family and so many others around the world, there are those with more severe, not readily apparent BAV/TAD variations. Given the prevalence of BAV in the population, their actual numbers likely are not small globally. What does the future hold for them? 

One of my greatest hopes for the future is that there will be the ability to proactively identify "the third man", those with complexities in their bodies that challenge the most skilled medical and surgical solutions. Yes, I continue to hope that in my lifetime, there will be greater understanding, more answers, and increasingly better treatment options than we have today. 

Best wishes for the New Year,

~ Arlys Velebir

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)

Disclaimer: This is my personal opinion only. It is information only, not medical advice, and does not represent the Bicuspid Aortic Foundation in any way.

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. 

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. However, medical literature states  there are only three 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 just three things. Perhaps it is not so much memory but focus that is the issue. 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. 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 classic "heart disease" that 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 

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, and 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:

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.

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."

We no longer live in the days of the brilliant Osler and Abbott, when autopsies confirmed aortic disease diagnoses only after death. There is an answer to finding aortic dissection in the emergency room in 2020. Is the answer still just randomly blowing in the wind, some victims of dissection getting diagnosed and having surgery while others lose their chance to fight for life?

~  Arlys Velebir