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