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.

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

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

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

Misdiagnosis and Disease - A BAV Perspective

Misdiagnosis and the "Big Three" Categories: 75% of "Serious Harms" , 50% from 15 Diseases 

Two papers published in the journal Diagnosis in July 2019 and May 2020 seek to shed light on the harm done by misdiagnosis of known diseases. These open-access papers are available online, as listed below (1) (2).  The authors identify three major categories and five diseases in each of them that are estimated to account for about 50% of serious injury and/or death: vascular events, infections, cancers. 

A Bicuspid Aortic Valve Perspective

Of the three categories, both the vascular and infection disease categories are of particular importance to those with BAV. Clinical significance of the bicuspid aortic valve , published in 2000, states that BAV may be responsible for more death and morbidity than all other congenital heart defects combined. Perhaps it is not surprising then, that the diseases listed include several vascular and infectious diseases known to cause injury or death in some who were born with BAV.

Diseased aorta in the chest

In the vascular events category the 5 diseases are:

• Aortic aneurysm and dissection
• Arterial thromboembolism
• Venous thromboembolism
• Stroke
• Myocardial infarction (heart attack)

In the infection category the 5 diseases are:

• Sepsis
• Meningitis and Encephalitis
• Spinal abscess
• Pneumonia
• Endocarditis

From the authors' conclusion: "We estimate that roughly one in 10 patients* with a dangerous 'Big Three' disease is misdiagnosed, and roughly half of those misdiagnosed die or are permanently disabled as a result." (1)  They go on to note that there is no indication that the error and harm rates are declining. They also mention there has been improvement in those diseases that have received actual sustained emphasis over several decades. 

BAV and These Diseases

Reading these papers I reflected on my late BAV husband's experience with 5 of the 10 diseases above at different points in his life, and the challenges we faced with diagnosis and treatment. He survived these serious threats to his life until intractable bacterial endocarditis ended it. His doctors marveled at how much he was able to overcome, which is the case with so many other "BAVers" I know.

Each person with BAV has their own journey. Seeking help may bring them to a physician's office, the
ER, perhaps both. Once there, even at the very first indication of an issue, will the disease be found in time to prevent disability or death?

What Can We Do? Be Informed, Be Proactive!

What can we do when we need help? We can be aware of these too often-missed diseases - the possible presence of an aortic aneurysm, the pain of an aortic dissection crisis, the potentially deadly infections - sepsis, endocarditis, or even pneumonia.

 With that awareness and realizing that these diseases may be missed, we can ask questions and whenever possible get additional opinions from the best expertise available. 

We can pursue answers,

 without embarrassment when discounted and misunderstood,

realizing the consequences to our future

if potentially deadly conditions are not found in time. 

Perspective from Sir William Osler in 1907 - Diagnosing Aneurysm of the Aorta

These diseases have long plagued humanity, and the challenges in finding them are not new either. The following words were written long ago by the renowned physician, William Osler, regarding diagnosis and aneurysm of the aorta (3), 

"The well-known dictum of Jenner may be taken as text: 'More mistakes are made by not looking than not knowing.' A majority of aneurism of the thoracic aorta present suggestive features to the eye, but the inspection must be made with care." 

Writing of having missed aortic diagnoses, Osler mentions an example of someone named McKinley. He continues with the often quoted:

"There is no disease more conducive to clinical humility than aneurism of the aorta."

Continuing, his words at the close of the page are compelling, where he quotes Pirogoff:

"Mistakes occur with the most careful and most skilful. Sometimes the diagnosis is beyond our art; more often it is not made because of the carelessness that so easily besets us in our work. The confession of the great Pirogoff always seems to me most touching: 'There are in everyone's practice moments in which his vision is holden, so that even an experienced man cannot see what is nevertheless perfectly clear, at least I have noticed this in my own case. An over weaning self-confidence and pre-conceived opinion, rarely a weariness, are the causes of these astonishing mistakes.'" 

It is over 100 years since Osler wrote about diagnosing diseases of the aorta and aneurysms, citing Jenner and Pirogoff (4).  The tools of modern technology have not solved the diagnosis challenges. The authors of these recent papers, several from Johns Hopkins where Osler came as one of the founders in 1888, are bringing much needed focus to the harms done when certain diseases are not found or not found quickly enough. May the transparency and humility of Osler, Jenner, and Pirogoff, coupled with courage in the face of deadly diseases, be inspirations today to overcome limitations and find mechanisms to reduce misdiagnosis.

The Challenge in 2021

Doug Grieshop's Diagnosis,
 BAV with Aortic Dissection/Rupture,
 Came too Late, During an Autopsy

Consistently diagnosing just a small number of diseases in time (aortic aneurysm and dissection, endocarditis, and sepsis among them), would drastically alter the ability of these diseases to disrupt and destroy lives, some of whom were born with bicuspid aortic valves. Someone with BAV has too often been discounted with "anxiety", sent home when a heart attack was ruled out. Some are disabled, some have died. 

The crisis eventually passes,

 but the scars linger.

May the new light these papers are shining

 be a springboard to improve diagnosis,

significantly reducing injury and death.

 ~Arlys Velebir,  Bicuspid Aortic Foundation

(1) Newman-Toker, David E., Schaffer, Adam C., Yu-Moe, C. Winnie, Nassery, Najlla, Saber Tehrani, Ali S., Clemens, Gwendolyn D., Wang, Zheyu, Zhu, Yuxin, Fanai, Mehdi and Siegal, Dana. "Serious misdiagnosis-related harms in malpractice claims: The “Big Three” – vascular events, infections, and cancers" Diagnosis, vol. 6, no. 3, 2019, pp. 227-240. https://doi.org/10.1515/dx-2019-0019

(2) Newman-Toker, David E., Schaffer, Adam C., Yu-Moe, C. Winnie, Nassery, Najlla, Saber Tehrani, Ali S., Clemens, Gwendolyn D., Wang, Zheyu, Zhu, Yuxin, Fanai, Mehdi and Siegal, Dana. "Serious misdiagnosis-related harms in malpractice claims: The “Big Three” – vascular events, infections, and cancers" Diagnosis, vol. 6, no. 3, 2019, pp. 227-240. https://doi.org/10.1515/dx-2019-0019

(3) Modern Medicine Its Theory and Practice, Volume IV, Diseases of the Circulatory System - Diseases of the Blood - Diseases of the Spleen, Thymus and Lymph-Glands, Chapter XI Aneurism,William Osler, MD, FRS, p472.

(4) The Life and Work of Nikolai Ivanovich Pirogov (1810-1881): An Outstanding Anatomist and Surgeon

*Bolding added to quotations through out.

BAV Lifelong Care - Carrie Mettler's Journey - 2016 to 2021

May 2021 - Life is good!

Background - Carrie's BAV Discovered in Early 1990's

Carrie was born with BAV, but she did not know this until she volunteered to be a guinea pig for a new echocardiogram machine in the early 1990's. There it was - a two-leaflet aortic valve! The next year, after her sister's diagnosis included not just BAV but an ascending aortic aneurysm, Carrie had another echo which showed that her ascending aorta was enlarged also.  

Carrie already had begun her heart journey with the implantation of her first pacemaker in 1989, when she was only 29 years old. In the following years, her pacemaker journey alone has required multiple interventions due to infection, broken pacing leads, and device upgrades. In 2014, open heart surgery was required to address complications caused by pacemaker leads! Carrie had developed Superior Vena Cava (SVC) syndrome, which is obstruction of the blood flow though the superior vena cava. The cause was old pacemaker leads located there and scarring that had developed in this blood vessel over time. During surgery, Carrie's own pericardial tissue was used to enlarge her right atrium and patch her superior vena cava, and the abandoned pacemaker leads were removed.

Here, we focus especially on the most recent five years of Carrie's journey, from 2016 to the present. Carrie began experiencing chest pain/discomfort that year, causing her to seek out the expertise of the Cleveland Clinic. The remainder of this post are are in Carrie's voice, with paragraph captions added.

October 28, 2016 - Chest Pain Prompts a Visit to Cleveland

So off I went for evaluation at Cleveland Clinic.  I flew to Chicago where my little sister, Theresa, picked me up.  We then took an all-day road trip to Ohio.  We spent several days there.  I had an echocardiogram, a CT, labs and EKG.  Once all the tests were completed we met with a cardiologist as well as Dr. Eric Roselli, a cardiothoracic surgeon.  Dr. Roselli viewed the CT result with us, where we were able to view the actual scan.  My aorta was stable and they all felt that it was reasonable for me to continue on with the watch and wait approach.  The chest pain issue was still unsolved so I just had to back off on my running and switch to walking.

 So, over the next several years I kept up to date with my local cardiologist, where serial imaging of my aorta and bicuspid aortic valve remained relatively stable.  Chest discomfort remains unsolved.

March 2019 - Diving Competition, Bee Sting and  Breast Cancer

During a dock diving competition where my little dachshund was competing I was stung by a bee on my left wrist.  Initially the sting was painful but I continued on with the day just feeling tired.  Then I developed a large localized reaction which spread up past my elbow.  After seeing my primary care provider he instructed me that if the redness continued going up my arm that I should go to the ER.  I did end up going to the Emergency Room where I was given appropriate medications and steroids.  The “cellulitis” finally resolved.  It was also deemed that I am allergic to bee venom.

Bee sting reaction!

But something wasn’t right.  I just kept feeling like I had an ache in my left armpit area for a while…then I started feeling pain in my left breast. 

I was watching a nightly national news program one evening and they aired a segment about women with fibrous breast tissue should be screened with 3D mammography.  After seeing this and knowing my mother passed away after a battle with metastatic triple negative breast cancer…I called my PCP and asked if he would order this mammogram for me. 

 I went in for the mammogram the end of April.  The next day my doctor’s office called to tell me that I had a suspicious lesion in my left breast and that I will be scheduled for an ultrasound.   After the ultrasound my PCP called me to let me know I needed to have a biopsy. 

The biopsy was done on May 2^nd.  Four days later, I got the call…You have cancer!  My knees buckled and I felt sick and so frightened.  I got my wits together and went out to where my husband was working in our yard.  It was all so surreal.  Right up there with when I found out I had a brain aneurysm.  All of the results came back, and I indeed had the same cancer that my mother had.  Triple negative breast cancer.

Cancer Surgery and Chemo

There were many appointments and consultations, then finally I had surgery to remove both breasts June 24, 2019.  I spent a few days in the hospital and had the support of my husband, Vic, my sister Theresa and several of my dearest friends.  I actually felt pretty well and was optimistic as no cancer was found in my lymph nodes and the tumor wasn’t too large.  But it was still considered a high grade tumor and I had to be scheduled for 16 weeks of chemotherapy.   Prior to this I had to have a pre-chemo echo…where everything appeared stable.

After discussing everything with my oncologist and surgeon they agreed that I could take the RV trip we planned to our favorite park on the Metolius River in Oregon.  I was very grateful for this time to reflect and be with my husband and my sister by heart, Judi and her husband Greg.  We were also joined by two more dear friends one of which was a retired nurse.  I was in good hands and I felt the trip to be restorative.  

January 2020 - Last chemo treatment

August 5^th 2019     I received my first round of chemo.  Judi was with me for every infusion from then on.  I developed most of the nasty side effects.  Anemia, neutropenia, oral thrush, nausea and hair loss.  We had to postpone several treatments until my labs rebounded.  I also had to have several blood transfusions. 

I was pretty much exhausted most of the time but pushed myself to walk my dogs as much as possible.    In October I had another echo, and my cardiologist told my aortic valve was now mildly stenotic.  Fast forward to December and the aortic valve showed moderate stenosis.  I became more fatigued and short of breath and even after chemo ended in January of 2020 I still could not regain my stamina.  My cardiologist recommend that I work out but I could barely climb a flight of stairs.

 March 2020 - COVID, Fatigue, BAV Severe Stenosis, A New Door Opens!

The Covid 19 Pandemic lock down started.  I was supposed to have the tissue expanders removed and proceed with breast reconstructive surgery.  This procedure was deemed elective and my case was cancelled indefinitely.  I still felt short of breath, and fatigued along with exertional chest discomfort.  Not to mention how uncomfortable the tissue expanders were. Then in May my cardiologist ordered another echo.  My aortic valve stenosis was now severe.  I had mild swelling of my ankles and was becoming depressed and very frustrated. 

One day in August, my husband and I were driving down our road and stopped in to see our relatives that were out in their yard.  This was one of those divine intervention moments.   You see, this family member also has severe aortic stenosis!  Two of us, living on the same road, with the same problem. We talked for a while, and she told me that she had become discouraged with her local cardiologists and finally went to UC Davis in Sacramento, where she felt she receives superior attention.  I told her that I was also frustrated with my care and that my cardiologist of many years was set to retire.  I asked her to get me some contact information the next time she went.  A few days later she stopped by with the TAVR (Transcatheter Aortic Valve Replacement) Nurse coordinator’s card and said “she knows you”!  I looked at the name on the card and couldn’t believe that it was someone I used to work with in a cardiology office where I worked for many years.  She actually helped train me in pacemaker and ICD follow up….and we even had golfed together!  She was expecting my call!  I felt so relieved and thankful! 

 September 1^st 2020 - BAV Needs Replacing, Stable Aorta

Carrie's aorta 
2016 at Cleveland Clinic

Judi picked me up and off we went to UC Davis, where I had another echo and was evaluated by the cardiologist specializing in TAVR. I was also evaluated by a cardiothoracic surgeon and many medical students.

They all agreed that my aortic valve needed to be replaced, but they needed more time to go over the echocardiogram results and the recent CT scan.  Because I had previous open heart surgery for SVC syndrome and also have an ascending aortic aneurysm, I was considered high risk.

I felt very comfortable and confident in my new care team, knowing they would take time to make a treatment plan. Two days later I received a call from the TAVR nurse.  They determined that I was a candidate for TAVR and that my aorta was considered stable. 

 October 5, 2020 - TAVR in Native BAV

I had a transcatheter aortic valve replacement (TAVR) using an Edwards Lifesciences Bovine valve.  Everything went very well and I felt the difference immediately!  My physicians are optimistic that with my new valve, some of the pressure will be taken off of my ascending aorta.  We will continue to monitor my prosthetic valve along with my aneurysm. 

June 2021 - Blessed in the Face of Adversity

Today, I feel very well!  I was finally able to have my breast reconstructive surgery… also at UC Davis.  In fact, I have transferred all of my care to UCD.  My care has been amazing!  Yes the past few years have been a challenge…as they have for all…but again, I would not change a thing.

Feeling blessed in the face of adversity is truly a gift…and I will always be thankful for the bee and the cow that helped save my life! 

Carrie has been with us at BAF since our beginning days,

 and it is our privilege to walk with her always. 

Thank you, Carrie,

 for once again sharing your courage and strength with the world,

and through each experience,

 Creating a Climate of Hope

~Arlys Velebir

                    Bicuspid Aortic Foundation

Also see Carrie Mettler - A Wonderful Athlete with BAV   and 

Carrie in 2016 - Seeking Opinions for a Complex Situation

I Need to Find Somebody Who Cares

Every Month is Heart Month, Every Day is Heart Valve Day

February 2021 is just a memory now, the calendar has turned to March. Heart Month 2021 and Heart Valve Day 2021 are over.

The need for accurate information, greater awareness, and compassionate care remains just as intensely, for it is always there!

The increased focus, still so fresh from recent February activities, can be the launch pad for maintaining focus and resolve to better meet the needs of those with BAV and associated TAD. Above all, at BAF we are reminded of the need to care, every month, every day.

"I Need to Find Somebody That Cares"

In 2020, the Bicuspid Aortic Foundation heard from a man, (I will call him Mathew, not his real name) who had been told a few months earlier that he had a bicuspid aortic valve. He was also told that he had an aortic aneurysm whose diameter was just under 5 cm. He mentioned several questions he had, as he pondered his active life and what options for surgical repair one day might mean to his future.

He closed that first message by saying that he felt quite lost as he worked through what these discoveries about his heart and aorta meant. And then he wrote, "I need to find somebody that cares."

A Day in April 2020 - Lost

It wasn't Heart Valve Day, it wasn't Heart Month, and it wasn't Thoracic Aortic Disease awareness month (which BAF observes in September). The COVID-19 pandemic was very real, but that is not what weighed so heavily on him, moving him to contact the Bicuspid Aortic Foundation.

It was a day in April, 2020, when this man, needing information, compassion, understanding, and hope, reached out across many miles and borders. There are no borders when it comes to this!

Above all, Mathew needed "somebody that cares". 

How do I know so well what Mathew needed? Experience. The myriad of emotions from the time when we desperately searched for information and help for a bulging aneurysm above my husband's heart are not forgotten as I write this twenty years later.

Not the least of those feelings were the icy tentacles of fear, not inappropriate when confronting a reputed killer. That icy fear began to melt in a surgeon's office, in the presence of accurate information, a plan for treatment, and from that moment forward, lifelong, compassionate care.

Yes, we had a great deal in common with Mathew, having never met him, on that April day in 2020.

A Day in March 2021 - Strength

Not quite a year later, in March 2021, what is it like for Mathew, who was lost and needed someone to care?

He was moved to reach out again, from a very different place. There is no mention of feeling lost, but rather a tone of strength. "I am doing well", followed by beautiful words of encouragement, which we all need so much! 

When this happens, whenever we move from fear and confusion to the place of informed empowerment, we have strength to fully live, making informed decisions for ourselves and sharing our strength and courage together.

How do we at BAF know this? Experience.

Mathew has done this, these recent months. 

Many others have done it too.

At BAF, we are here to walk with you, whenever you would like.

Moving from fear and confusion 

to accurate information,

being in touch with those who

 understand and care, 

Living our lives,

Creating a Climate of Hope,

~ Arlys Velebir

                        Bicuspid Aortic Foundation

Pursuing Answers in Memory of Those We Have Lost - Heart Month 2021

Red Jasper

 On this first day of February, designated as Heart Month 2021, at the Bicuspid Aortic Foundation we begin by remembering those we have lost, for it is in remembering them that we are confronted by one of the most compelling reasons we exist, the persistent, tragic loss of life, too often in the young. Despite advances in diagnosis and treatment, we continue to lose them. We begin by remembering some young men in their 30's.

Forever 33, Almost 34, in 2020

Michael Anthony Smith was born in August, 1986. Just under a week before his 34th birthday, in August 2020, he passed away in a health center in Gainesville, Florida. 

Michael's memorial page, including a video tribute, are located here. 

Those who visit the link above and watch the video will recognize a strong, active, vibrant young man. There are no words to adequately convey the tragedy of his loss, the pain and emptiness of those love him.  

Forever 33 in 2004, Forever 34 in 2012, Forever 30 in 2013

Over the years we have heard of other young men who succumbed unexpectedly to BAV/TAD complications. Doug Grieshop turned 33 the day before his death in 2004; an autopsy revealed a ruptured aorta and an undiagnosed BAV.

Chuck Doherty was 34 that day in 2012 when he collapsed in the driveway of his home as he returned from a run; Chuck's BAV and aortic stenosis had been diagnosed, but that was insufficient to protect him from sudden death.

Chad Rogers was 30 when he failed to return from his run on Monday evening, July 22, 2013. His body was not found until Friday afternoon. Chad's BAV was known since infancy, but similar to Chuck, his physical activity had not been restricted. 

Doug Grieshop's family shared his life initially here, and there is more in this follow up, Forever 33.

Chuck Doherty is remembered here.

An account of Chad Rogers' cause of death is here.

There are others, also young, also athletic, also suddenly taken, whose families have come to us after their loss. As I type this, I remember an autopsy report sent to BAF years ago; it described the examination of a healthy young man's body. He had dropped dead while playing, if I recall the sport correctly, volleyball with his friends. The report detailed his body, healthy and normal with just one exception. The only finding was a bicuspid aortic valve; despite CPR immediately, his heart could not be restarted, his death perhaps due to an abnormal heart rhythm. 

And so we begin Heart Month 2021 remembering this group of young men, healthy appearing, athletic, vibrant, often in their 30's. 

It is approaching 17 years since Doug Grieshop died, and in the intervening years other young men in their 30's have died also. How many, we do not know. How can we see beyond their glowing, healthy appearance and identify those at risk of a deadly event (aorta, aortic valve, arrhythmia) before they are prematurely taken, leaving loved ones to go on somehow, deprived of their gifts, energy, and  many contributions to our world?

In their memory, we pursue the answers,

in February 2021, 

Creating a Climate of Hope,

~ Arlys Velebir

Bicuspid Aortic Foundation

TAD Awareness September 2020 - More Awareness, Many Challenges Today

On this last day of September 2020, where are we in terms of awareness of aortic disease in the chest, both for the public and for medical professionals? 

Are We Still Learning "the Hard Way"?

Once the aorta threatens or takes away someone's life there is painful "awareness". It is a "hard way" to learn about a potentially deadly condition. For physicians, missed or delayed diagnoses and possibly loss of a patient are also learning "the hard way"; that is, if the diseased aorta is actually ever discovered at all. It is possible, in the absence of autopsies, that deaths due to aortic disease in the chest remain hidden, uncounted.

Would it Be Different Today?

At the time Michael Kirk went to the ER with symptoms, he was a cardiac intensive care nurse at that very hospital.  No one thought of his aorta, although over an agonizing 68 hours, they thought of many other things, including anxiety! The medical team ultimately learned from Michael, and he survived the crisis.  Michael shared his experience with BAF here. Thinking of Michael's experience, would it be better in 2020? Would his family members, assumed to have died suddenly of "heart disease" be checked for aortic disease today?

In 2020, Someone Still Needs to Think of the Aorta in the ER

There is a marker in the blood, troponin, that indicates heart muscle injury (typically signs of a heart attack) that appears to be broadly understood and used. There is no simple equivalent for the aorta today. Currently, someone needs to think of the aorta, to rule it in or out with imaging, as one of the major killers in the chest.

 However, a paper published in July 2020 offers hope for additional diagnostic help in the future. Imaging and Biomarkers in Acute Aortic Syndromes: Diagnostic and Prognostic Implications :  "It is expected, in the near future, the development of serologic and imaging biomarkers able to early detect clinically-silent pathologic changes in the aorta wall before (primary prevention) and after (secondary prevention) the acute index event."

This is hopeful news! However, checking for these biomarkers would still require that someone thinks of the aorta, not just the heart!

This court case is an example of death after being sent home from the ER, when the aorta was not considered. 

Richard Houchin, whose aorta dissected in 2004,  remembers hearing someone say "aorta" while in the ER, and thinking fuzzily, what is my aorta? Richard had a number of things "go right" that day, beginning with the paramedics who thought of his aorta, despite instructions from a major medical center to treat him as a heart attack victim.

When it is not a heart attack, we can help physicians think of the aorta in the chest by bringing it up to them.

In 2020, Someone Needs to Care for Aortic Dissections Survivors

An article published this September has this poignant title: 

Am I going to die now? Experiences of hospitalisation and subsequent life after being diagnosed with aortic dissection

The full article is not freely available to the public. However, as the abstract indicates, this is an effort to understand and improve the experience of those who survive aortic dissection. 

In 2020, Someone Needs Answers and Support When Thoracic Aortic Disease Causes Sudden Death

There are no words to describe the devastation left behind among the living who suddenly lose a loved one. 

How many times is aortic disease in the chest the actual culprit, not a "heart attack"? 

Amy's family had the courage and resources to arrange for a private autopsy, which unmasked the undiagnosed BAV and diseased aorta that abruptly took her life. Here is Amy's story.

In 2020, Focus is Still Needed on the Aorta, Not Just the Heart

For those with BAV, they may only be told about their aortic valve and heart. I recently spoke to the mother of a wonderful, active boy, age 14. While hospitalized at a major medical center for another reason, the presence of a bicuspid aortic valve was discovered. The initial information given was not overly concerning. When following up with a local cardiologist later, however, this boy and his parents were shocked to be told that his aorta was enlarged, and his physical activity should be limited. 

How could that be possible? His aorta had not even been mentioned, and all of a sudden, it is an issue important enough to restrict his sports participation? This is devastating to anyone, let alone an active, strong teenager.

It was not easy in my own family when a large ascending aortic aneurysm was "discovered", years after being told all was safely fixed following BAV replacement surgery. Later we learned the aorta was already enlarged at the time of the BAV replacement and had quietly continued to grow! 

No, it is not easy to work through the emotions and lifestyle changes that may follow abruptly learning about an enlarged/aneurysmal aorta. Acceptance of physical restrictions and lifechanging adjustments are not trivial for those whose bodies are seemingly so healthy, often naturally gifted athletes who are typically involved in numerous activities and sports. 

Parents, families, and each one with BAV deserve compassionately shared accurate information about their aorta along with their BAV/heart, ultimately enabling them to live with confidence, supported by their medical care team.

The following paper published in 2020 provides international information from multiple medical centers, regarding 2,122 BAV children; half of them had an enlarged aorta. Their average age was 10.2 years.

Predictors of Bicuspid Aortic Valve-Associated Aortopathy in Childhood: A Report From the MIBAVA Consortium

Keeping Our Focus on the Aorta Every Day, Every Month, All Year Long

At BAF we have been observing September TAD Awareness for a number of years. We find in 2020 that the aorta in the chest still presents many challenges. The various efforts to raise awareness this year all help the public understand the importance of their aorta, this large candy-cane shaped artery that begins with their heart. May we maintain this focus every day, every month, all year long. 

 On this last day of September,

 we can help everyone we meet

to remember the aorta and

seek accurate information,

empowering them to live confidently,

 Creating a Climate of Hope,

~Arlys Velebir

                         Bicuspid Aortic Foundation

Thoracic Aortic Disease Awareness September 2020 - TAD in the Midst of COVID-19

 How many have been injured or died from a diseased aorta in the chest (thoracic aortic disease (TAD)), not receiving treatment for various reasons during the COVID-19 pandemic?

Did the pandemic come between those with a torn aorta in the chest and the emergency physicians and skilled surgeons who could save them? It is reasonable to believe it did. The following is written by physicians in New York:

The COVID-19 Pandemic and Acute Aortic Dissections in New York

A Matter of Public Health

In the above, a "significant and precipitous drop" was identified in the number of surgeries for acute aortic dissection. The best hope for someone with acute type A dissection, which involves the ascending aorta and strikes without warning, is coming to the ER, being diagnosed, and receiving emergency surgery. How many were denied that opportunity due to the pandemic?

As the above concludes:

"It is critical, as we adjust to the pandemic, to balance the public health imperative of social distancing with the individual need to consult in the presence of sudden severe symptoms. Furthermore, additional resources, ranging from telemedicine to numbers of first responders, should be greatly increased. This serves as a word of caution for cities yet to experience a surge in COVID-19 cases, as well as for future similar events."

Inspiration and Also A Warning

The following is an account of a professor from Idaho who traveled to New York for life-saving complex aortic surgery during the pandemic. It is hopeful and inspiring, while at the same time containing a warning about the vital importance of lifelong monitoring of the aorta in those born with bicuspid aortic valves.

BACK IN BUSINESS: CEED Director undergoes life-saving heart surgery in New York amid pandemic

One of the surgeons who saved Jeff Street, pictured with him in the article, is Dr. El-Hamamsy, who is also among the authors in the first article above. He is among the skilled surgeons that could have helped those living nearby also, if only they had reached him.

Regarding the warning in this man's experience, it is important to avoid unnecessary, life-threatening aortic dissection or rupture in the first place. How did this man's aorta reach this very fragile state undetected? 

Jeff Street had surgery to address his bicuspid aortic valve 23 years earlier. Presumably he had cardiac care over those years, but what about his aorta?

Only when he became symptomatic this year, the article states, was an aneurysm "discovered". More concerning still, was the actual condition of his aorta when fully exposed during surgery on June 9th (quoting from the article):

" “Absolutely it was a life-saving surgery,” Street said. “I did not know that my aorta already had a small leak on it. The only thing holding it together was the scar tissue from my previous surgery 23 years ago.”"

Words like "miracle" come to mind as one reads about this man's experience - fragile tissue somehow holding together until he was in the hands of a surgeon with the skills to save his life.

And then, thoughts turn to those whose lives have ended abruptly, unknown and uncounted.

For those in BAV families, aortic valves are unquestionably important and vital. It would be beyond a tragedy to provide state-of-the-art treatment and care for their aortic valves, but not their aortas. 

This September 2020, once again it is important to raise awareness of the aorta in the chest in those born with bicuspid aortic valves and their family members.

In the midst of  this pandemic,

We again highlight the aorta in the chest, 

Raising awareness

and  

 Creating a Climate of Hope,

  ~Arlys Velebir,

                           Bicuspid Aortic Foundation

 

 

 

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