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 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

BAV, Aorta 4.5 cm and "Paper Thin"

Surgery is Behind Him Now!

Hope, Courage, and
 the Heart of the Warrior (red jasper)

With the soft chiming of a monitor as background music, his voice on the phone was music to my ears. From his hospital room he told me some wonderful things - he is recovering well, he has a new prosthetic valve in place of his failing BAV, and lots of his aorta was removed. He will soon be out the door and on his way home to recover, wonderfully repaired inside!

And then he told me something that gave me chills. His surgeon had found that his aortic aneurysm was paper thin. Yes, paper thin and fragile at "only" 4.5 cm.

In theory, it was not supposed to be that thin, fragile, and dangerous at that size. In some people, it is.
The challenge is, who are those people? How can they be identified?

His Failing BAV Justified Surgery

He contacted us at BAF after his initial diagnosis. He had additional testing and then sought a surgical opinion at a major center. The surgeon there initially thought of retesting again in 6 months, but after further reviewing the failing BAV and its affect on his heart, the decision was made to schedule the surgery.

Did a failing BAV prompt surgery

 in time to prevent his aorta from tearing? 

Yes, the known justification for his surgery was the BAV. That is because there is no way to know, no testing, to show how fragile the aneurysm is. Size alone does not tell enough.

There is no current testing to tell anyone that this aneurysm was dangerously thin and fragile at a relatively small size.

This is why even those who are diagnosed may dissect or rupture their aorta before they have surgery. One surgeon told me that a local hospital is actually seeing more, rather than fewer, dissections come to the emergency room. Something is very wrong with that picture! I am so thankful that it will not happen to this young man -  he will not experience life-threatening bleeding in his chest. Some time in the next 6 months, without surgery, what would have happened to him? That question, that threat, for him, is gone now.

He is 39 years old, a husband and father with young children. He is the typically active BAVer. He holds a responsible job and is intelligent and highly capable. In short, he is in the prime of his life.
Thanks to timely diagnosis and surgery, he will soon resume his active, busy life with his family once again.

Prevent Dissection and Rupture - They Should Not Happen!

Dr. Lara Gharibeh, Dr. Alice Lau
Both engaged in BAV research at U of Ottawa

This is why research that helps explain what happens in the walls of the BAV aorta is so critical. If markers can be found that will show which aortas are at high risk of tearing(dissection) and rupture, those people could have surgery before it happens.

This is why the recently published work about the BAV aorta at the University of Western Ontario is so important.  At the University of Ottawa, we look forward to the results of research on the aortas of BAV mice.  We are encouraged that there is an increasing awareness of this issue, and there are those pursuing greater understanding, such as the authors of this recent paper about BAV aorta risk stratification.

With Dr. Marc Ruel, Chief of Cardiac Surgery, Ottawa Heart Institute

The treatment for BAV and ascending aneurysms at this time is primarily surgical. While we wait for more answers, being proactive in searching for information and answers, consulting with knowledgeable surgeons at aortic centers, and making proactive decisions together, are all things that we can do to achieve the goal: safe, elective surgery. This young man has had his elective surgery now! He has our very best wishes as he returns home!

Asking questions,

Seeking answers, 

Together we are,

Creating

 a 

Climate of Hope,

~Arlys Velebir

        Bicuspid Aortic Foundation