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.

The Power of Hope and "Redo" Aortic Surgery

Introducing Joseph
It was 10 years ago. We were two voices on the phone together. We have never met in person. I have never seen his picture. None of that matters, not then and not now. What mattered was what we said to one another.

His name is Joseph. Heart surgeons refer to someone like him as a "redo".

His daughter had put us in touch. He was already a survivor of an aortic crisis, having come through aortic dissection and ascending aortic aneurysm surgery. Now his bicuspid aortic valve had become calcified and narrow. He was facing another major open-heart surgery, not so very long since the last one.

Yes, surgeons call someone like Joseph a "redo". I had heard some things about "redo's", such as the challenges of scar tissue that can make even opening up the chest safely a challenge.

An example of BAV Stenosis
This was not Joseph's valve!

How could I help Joseph? 
By this time, I had already been through enough in my own family to know about facing hard things, including more than one open-heart surgery, because of BAV and the aorta.  I also knew how much strength and ability to heal those with BAV often have. They are generally not "frail" people, and as Joseph told me about himself and how active he was, I recognized that strength in him.

He had already come face to face with death when his aorta tore. He had come through major surgery to replace part of his aorta. Now it was his stiff, calcified BAV forcing intervention. Before open heart surgery and artificial aortic valves, the end state of aortic valve stenosis was heart failure ending in death. There was no question the stenosis could not be allowed to weaken his heart and end his life, when a surgeon could help him. He already knew these things from his doctors. I must leave to them all the challenges of a "redo" surgery.

Joseph didn't need information from me. 

He didn't need any explanations either.

He needed hope.

"Am I going to make it?"
I am so glad that Joseph felt comfortable asking me this, the life-and death question that weighed heavily as he chose to have surgery again:

"Am I going to make it?" 

Across the years, I hear once again the sound of his voice as I write this.

"Yes, you are! And we will talk again!"  

And talk again, we did! 

What a moment, hearing his voice again!

 Yes!!!

There is nothing like it! 

Courage

Red Jasper
The Warrior Stone of
Strength and Courage

Fear is normal; we all have fears to face when entering a hospital and having such major surgery. Courage enables us to go forward, despite our fears, and receive the help being offered. Joseph was a very courageous man. It was my privilege to support his courage, knowing that others had come through multiple surgeries like this.

I have sometimes used the analogy of surgery being a doorway or a gate; something we go through and there is our life once again, the diseased valve or aorta that plagued us left far behind.

A decade has passed. Joseph was not a young man back then. Age in itself is not a limitation to receiving help! How beautiful to think of  him continuing forward and having the precious time since then.

Hope
Thinking about Joseph today inspires and moves me to share this experience with others. There are others facing yet another surgery because they were born with a bicuspid aortic valve; others needing hope and support of their courage today.

Perhaps I should mention that my late husband had a total of four open heart surgeries over 27 years. Four precious times when I could keep my promise to welcome him on the other side of the surgery door, as he awoke from anesthesia.

There is so much that is possible today, while we look forward to an even better future. TAVR technology has continued to improve, emerging as an option to be individually considered in cases of both native and prosthetic aortic valve stenosis. While we must continue to speak up about our needs and the things that must be improved, this hopeful "climate" surrounding us is central to the Bicuspid Aortic Foundation's mission.

Joseph and I are sharing

this especially with you,

As you bravely choose and prepare for

 another surgery,

 "Creating a Climate of Hope".

~ Arlys Velebir

                           Bicuspid Aortic Foundation

Continued Thoughts and Prayers for Adam

Adam was slated to have surgery last week.

Plans sometimes change due to circumstances beyond our control.

That is what happened to Adam.

He is now scheduled for surgery early Monday morning.

Our thoughts and prayers are with him, his family,  and his surgeon. 

The Three W's: What, Where, When

Coming to grips with having open heart surgery is a process, except in emergency situations. Adam had done this once before, when the aortic valve he was born with and the bulging aorta above it, needed surgery. That is how we at BAF first met Adam, as he researched information as part of his decision making process. 

This time, with infection in the picture, surgery needed to be done promptly to fully vanquish the bacteria and put a new, undamaged aortic valve in place. When was to be last week. Where was also settled - Adam lives in an area large enough to have not only an infectious disease specialty center, but also a skilled aortic surgical specialist. Peace with the What (mechanical, homograft, Ross procedure, bovine, or porcine for the new valve) also finally came after weighing the choices carefully.

Valve choice decisions, so many years after the first prosthetic valves were implanted decades ago, remain a thoughtful, individual decision based on many factors. As I heard many years ago now, there is no perfect choice! That is true, but there are many good ones.

When infection enters the picture, as it has for Adam, the considerations are altered. The risk of life-threatening infection returning and destroying the new valve weighs heavily, along with other factors, on the decision-making scale.

Ready for Surgery - Let's Get This Done!

With the help of his physicians and his own reading, Adam was at peace last week and more than ready very early the morning of surgery. By 6 AM he was prepped for surgery, partly sedated, and then . . . . he waited. And waited some more and some more . . . .

Finally, as morning faded and turned into early and then late afternoon, plans were abandoned for Adam to have surgery that day. His surgeon had been working to save someone else all those hours.

Adam says that whoever the other person was, they needed the surgery more than he did.

Adam's Turn - Monday Morning, June 10th

Adam will be ready once again, very early Monday morning. May it be his turn this time, since no one wants any complications to hurt him before the damaged valve with vegetation still clinging to it and any other remnants of infection are safely removed during surgery.

The plan is that Adam will have the Ross procedure. He has a skilled surgeon who will do all that is needed during surgery to clear the infection and save Adam in the best possible way.

Adam

Adam is a strong man, muscled and physically vigorous, with lots of energy! He is a loving husband and father, very much loved and needed. He is still young, with so much to accomplish.

How could it be that he has been so attacked by infection? He does not know how it got into his blood. Once finally diagnosed, he found that he was not alone, that there were so many at the infectious treatment center with their PICC lines in place, being treated for dangerous infections. 

Looking back, he says that, knowing what he knows now, with the first fevers he would have gone to the hospital and requested a blood culture be drawn. It is important for all with prosthetic or abnormal (bicuspid aortic) valves to realize that infection in the blood stream is a formidable enemy that does not play fair. The sooner it is found and treated, the better!

We join together in support of Adam and his family, 

asking that he have a safe, successful surgery, 

and that Adam will soon be home again, 

restored and infection free.

~Arlys Velebir

                           Bicuspid Aortic Foundation

Our Thoughts and Prayers for Adam

Red Jasper
The Warrior's Stone

It is special to hear from someone again after many years.
Sometimes it is good news, just checking in to say how well it is going.
Sometimes it is far from good news, it is heartbreaking.
That is when we are reminded why BAF exists.

I spoke with Adam today. He had his aortic valve and aortic aneurysm replaced in 2006. He was so young when he had surgery, just 30 years old. A checkup last November showed his prosthetic bovine aortic valve, over 12 years old by then, still holding up well.  He and his wife have a lovely family of four children. Life had been good!

And then without warning, everything changed.

Infective Endocarditis (IE)
 Beginning in early January, Adam ran a fever and did not feel well. He went to a doctor, who prescribed an antibiotic. It did not help. He continued to run daily fevers. He saw a second doctor, then a third. The medicines prescribed just did not work.

Finally in early April, three months from the first onset of fever, a fourth doctor diagnosed the problem: infection in his heart valve, endocarditis.

He has finished a course of intravenous antibiotics and now faces surgery. There is a question about the best option to replace the damaged bovine valve. There is some controversy that makes the choice difficult. The reason is that once the heart has been infected, there is an increased risk of getting infection there again.

Yes, infection changes the picture, because infection in the heart may be deadly. No one wants another infection there, and it dominates the thinking and planning for surgery.

Bacteria in the Blood
How did Adam get this infection in his heart? He does not know how it happened. Maybe a small cut or break in his skin. Somehow, the bacteria entered his blood. As his blood washed over the bovine aortic valve surfaces with each heart beat, some of the bacteria "stuck" to the valve surfaces.

Over time the bacteria grew entrenched on the valve, damaging the leaflets, forming clusters called vegetation. The bacteria may also hide in crevices that are not visible or easily reached by antibiotics. Surgery is aimed at addressing all of these issues.

Endocarditis Increasing
While many things may have been conquered or at least reduced with time, endocarditis is not one of them. It is actually increasing according to medical articles such as the following.

Endocarditis: An Ever Increasing Problem in Cardiac Surgery

Current practice in prophylaxis of endocarditis: are we running into trouble?

Only the introductory text is available to the public, but these example articles speak to the increased risk of infection in the heart.

Cleveland Clinic has an endocarditis center, where more information is available.

Thoughts and Prayers for Adam and his Family
Adam is in the midst of working through the three W's: What, Where, and When surgery will be done. Surgery must not wait because of the vegetation still in his heart, so the what and where need to be settled quickly.

At the same time, this beautiful family has just learned that their little daughter, age 2, has leukemia.

Yes, this father, his little daughter, and this entire family will appreciate positive, loving thoughts and prayers today and in the days ahead.

Surrounding them

 with positive thoughts and prayers,

we Create a Climate of Hope.

~ Arlys Velebir

                           Bicuspid Aortic Foundation

*Originally published under the name Aaron, BAF now has permission to use his actual first name, which is Adam.