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