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

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

 

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

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?
Amy began this year, 2016, as we all did, with plans and hopes for her future. They were never to be realized, because she had been born with a bicuspid aortic valve, and over time an aneurysm developed. Today her voice is silenced. Who will speak for her, and the others we lose? 


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.

In giving a voice to Amy,
And others we have lost,

May we help the living
And in their memory, 

Create a Climate of Hope.

- Arlys Velebir