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Thursday, September 1, 2022
September Awareness 2022 - Thoracic Aortic Disease - "Gradually, then Suddenly"
Monday, February 7, 2022
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
Thursday, May 12, 2016
What Happened to Amy?
This is Amy's story. Only her name has been changed, to protect privacy.
Where is Amy?
"It's just not like her, Amy would have let us know if she couldn't come!" And so a "welfare check" was requested. Her home was silent. There was no response to the doorbell, the knocks, the calls of her name. Something was terribly wrong!
They found Amy inside her home, lifeless. Apparently there had been no opportunity to even call for help.
What Happened to Amy?
Her family members were notified. The police arranged for her body to be taken directly to a local mortuary. Deeply shocked, her adult children needed answers. Their mother had been an active, independent, and apparently healthy woman. She did not even have high blood pressure. They thought she would be with them for many years to come. How could this happen, without warning?
They needed answers.
In the place where Amy lived, no one offered to find those answers. After all, there was no evidence of a crime, no villain for the police to pursue. The death was clearly due to "natural causes". But, why? Is it really "natural" to die like this, without warning, when someone is so healthy?
Why Did Amy Die?
Amy's daughter told me that not knowing was unbearable to her family, much worse than the thought of an autopsy would be. Despite their tremendous shock at her sudden death, Amy's family found the strength and the financial resources to request a private autopsy. The local medical examiner was not available, and so Amy's family found their way to someone they describe as special, a compassionate, caring physician who carefully examined their mother's body and helped them understand. What that physician found inside would have been a gruesome sight.
Quite simply, Amy bled to death. Unknown to her, she had an aortic aneurysm in her chest just above a bicuspid aortic valve in her heart. Because there was an autopsy, Amy's death certificate is accurate, stating that death was caused by the rupture of an aortic aneurysm in the presence of a bicuspid aortic valve (BAV).
Courage to Press for Answers
Sometimes families cannot bear the thought of an autopsy on their loved one. There also may be veiled opposition, if the death occurs in a hospital, from a medical establishment that fears malpractice lawsuits. Sadly, the days of Dr. M.E. Abbott and Sir William Osler, who learned from autopsies and published extensively about aortic disease in the chest, are gone. As with Amy's family, it is the survivors' legal right to have an autopsy, but they may have to pay for it.
Knowledge - a Gift of Life
Amy's family was left with the sad, painful task of sorting through her things, closing her home. She was an avid reader, and they found many articles about keeping healthy, preventing disease. Among them may have been the usual warnings about preventing stroke and "heart disease". Like many with BAV, Amy's arteries in her heart were perfectly clean. She was not at risk of a heart attack. Sadly, there was nothing about BAV and aortic aneurysm, the condition that suddenly robbed her of life. Amy would not have even heard about what snatched her life away. If her family had not arranged a private autopsy, the reason for her death and the potential threat to their own lives would never have been known by anyone at all.
Knowledge like this comes at an unspeakable price. It is a price too high, too great to pay. It should not happen. There is a well known medical center not far from where Amy lived that could have performed her aneurysm surgery. They do this surgery frequently, perhaps almost every day.
Amy's blood relatives are educating themselves, being checked for BAV and/or aortic aneurysm. No one needs to tell them why now.
They know.
The Uncounted
The Bicuspid Aortic Foundation remains extremely concerned about the uncounted deaths due to BAV and aneurysm complications. Amy could easily have been one of those uncounted deaths, if her family had not had the courage and ability to seek the truth. Sudden deaths are too often simply recorded as due to natural causes, perhaps assumed to be due to a heart attack, but without any proof.
The lack of understanding and recognition of aortic dissection/rupture deaths in the presence of BAV, as well as sudden aortic valve related deaths has consequences not just for those who die, but for the living.
Other blood relatives may be at risk. But there are also consequences for the entire BAV population. Because these people are uncounted, they are not found in the statistics, not included in the medical papers published about patients who receive treatment. How can we understand those most at risk, those who may never even reach a hospital, if we don't learn from them? We don't even know why they died! And so, the statistics upon which treatment of the living is based can not include these unknowns, perhaps the most vulnerable, the most fragile. Must they continue to die?
Who Will Speak for Me?

At the Bicuspid Aortic Foundation we can help give them a voice and bring into focus these most vulnerable. We are enlisting the aid of pathologists to do so.
They are the physicians who speak for the dead, and in doing so, may save the living. We are also encouraging families where sudden death has occurred to take courage and inspiration from Amy's story, and seek the answers they need.
And others we have lost,
And in their memory,