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

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

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 2019 - A Virtual Walk with Bob Gies

An actual walk with Bob would be a fast one! Like many with BAV/TAD, his entire life has been on the go, pushing to the max, always at high speed!  

Ready for a ride, September 2019
A typical ride is at least 12 miles, sometimes up to 20

He agreed to pause and share this virtual walk with us, where we learn that despite having just celebrated his 75th birthday, Bob is still going strong! Ten years ago, he wasn't sure he would see this day. And if he was still alive, would he still be doing the things he loved?

A Gifted Athlete with a Heart Murmur

Bob remembers hearing doctors talk with his parents when he was very young, talking about his heart murmur. When he was ready for his freshman year of high school, more than anything else, he wanted to play football. A physical was required, and he still remembers what the doctor said to him, "You have a heart murmur, kid. But you're a healthy specimen, you'll be all right!"  Yes, the doctor let him play! Bob remembers thinking that this heart murmur must be something very special. Looking back, he never had any vision of ever having  a serious heart problem, not ever! 

Football in the Fall, Track in the Spring

In addition to high school football, which he played all four years, Bob excelled at track. He was a fast sprinter, winning both the 100 and 220 yard dashes, and also ran the low hurdles.  His junior year, at the state track meet, Bob scored more points than the rest of their team combined. A sprained ankle kept him off the track his senior year.

Somehow, Bob seemed to be just made for football. He says that great runners get all the glory in this game. Fast on the track, Bob was fast on the football field as well, thrilling the crowd each time he broke loose and raced down the field for a touchdown! Pointing out that avoiding being caught and tackled was an extra incentive, Bob says "If I ever got two steps ahead, they could never catch me."

Championship Game 1963

Bob is number 24

In the fall of 1963, his senior year, Bob describes the Huskies as a terrific football team, with lots of good blockers, everyone working together. A newspaper clipping from that time mentions that the backfield - Gies, Koontz, and Becker - was possibly one of the greatest combinations in the history of any Class C school. They were the dream team! What happened that year is well remembered to this day. They went into the final, championship game undefeated. They were also determined to always have the lead, to never fall behind, just as they had done all season! The game started with a kick off to the opposing team, who pushed them back to the 30 yard line. On their first play, Bob broke loose and carried the ball all 70 yards - touch down! That first play set the tone for the entire game. They were ahead from the start, and they stayed ahead, all the way to victory!

Greatest Of All Time (GOAT) Football Record 1963

Athletic Hall of Fame
Honors for Bob
 in 2016

Bob returned to his high school to be named to their Athletic Hall of Fame in 2016.

In 2018, a local paper wrote  about some of the football history for his high school.

There is Bob's name, along with two of his team mates, Koontz and Becker, on the GOAT list.

Bob still holds the record for rushing more than one mile his senior year and scoring 136 points that season. He did that while playing both offense and defense.

Bob on Sheep Mountain
About 30 Years Ago
3.5 hours to ski up,
 a fast 40 minutes back down!

Outdoor Sports After High School

Bob turned to outdoor, individual sports after high school: cross country skiing, biking, fishing, and generally enjoying the outdoors under that big Montana sky! There was no reason to remember that "heart murmur" of his youth!

He felt he was slowing a bit, fatigue and naps entered the picture at around age 60. Read here how his bicuspid aortic valve and aneurysm were discovered and about the surgery that followed. 





September 2019

This September finds Bob still busy in his bountiful garden, which he describes as a lot of work and great exercise!

He maintains his weight within a pound or two of what he was in high school. His step counter averages around 6,000 a day, with a peak of 24,000. Looking at his number during the day motivates him to get busy and do more! And then there are the miles on his bike, the miles on skis. . . .

It is just over 9 years since Bob had his life-saving BAV and aneurysm surgery. His bovine aortic valve and aorta are looking good at the nine year mark!

When asked how life has changed at this age, Bob says he misses pushing his body to the absolute limit, going flat out to the wall . . . his body just won't let him do that anymore!

His lifelong craving for exercise is still there, however. Exercise still makes him feel good!

Thank you, Bob, for sharing your life

on this walk with us,

Inspiring us, 

and

Creating a Climate of Hope,

  ~ Arlys Velebir,  BAF

                       

TAD Awareness 2019 Virtual Walk with BJ - TAVR to the Rescue

BJ Sanders shares her life story
on this virtual walk,
raising awareness of valve-in-valve TAVR

As you walk along with BJ, she begins with her life at age 12. Before your walk is over, she will tell you that today she is counted among those with a valve-in-valve TAVR and a grateful, happy heart!

1966 - A hint of what is to come

At age twelve my connective tissue disorder was emerging in the form of a severe scoliosis requiring six years of orthotics and finally culminating in a surgical procedure, Harrington Rod spinal fusion to straighten my spine at age twenty three. Despite the scoliosis I led a very active and healthy lifestyle!

1976 - Emerging labile hypertension

This is sometimes associated with Bicuspid Aortic Valve (BAV).

1981-1986 - Premature deliveries of my children

Once again connective tissue issues appeared in my three pregnancies. As a result of undiagnosed and misunderstood heart issues, I was placed on long periods of mandatory bed rest. Despite the bed rest, I had three premature deliveries.

1998 – No worries!

Despite multiple connective tissue concerns I had a very full and active life free of worry.

However, at this stage in my life I developed a prolapsed uterus, urinary incontinence and a rectocele all requiring repairs.

2000 – Searching for answers

Arrhythmias and visual concerns led to two echocardiograms, which the internist said were of no concern! Due to financial/insurance concerns, I requested my own copy of my medical records and noted that my aorta appeared to have expanded between echoes. I no longer trusted my internist!

Thus I began my long arduous Bicuspid Aortic Valve (BAV) and Thoracic AscendingAortic Aneurysm (TAAA) journey.

Over a span of two plus years, I searched earnestly for accurate knowledge to lead me to a physician with medical expertise in BAV and TAAA. There were many obstacles along the way, but the stars aligned the day I met Arlys Velebir in cyberspace. This eventually led to my introduction to Dr. Sharo Raissi, MD and the subsequent formation of the Bicuspid Aortic Foundation.

2002 – Watching and waiting

I entered into the watch and wait period under Dr. Raissi’s expert care. My quest for answers and understanding of my ascending aortic aneurysm and BAV were always met and my fears melted away knowing that I had found the expertise and compassionate care for which I had longed. HOPE had overcome FEAR!

 2013 – Time for Surgery!

Thirteen years into the BAV journey brought unexpected chest pain associated with a significant size increase in my ascending aorta. Dr. Sharo Raissi performed a TAAA reconstruction, a bovine artificial aortic valve replacement due to calcification of my BAV, and closed the left atrial appendage via a clip. Closing the left atrial appendage eliminates it as a potential source of embolic stroke in the future.

When I awoke from the surgery I distinctly remember Dr. Raissi saying, “In 10-15 years when this valve needs replacing, Transcatheter Aortic Valve Replacement (TAVR) should be available in the United States!” He also explained that he put in the largest artificial aortic valve possible for me; so, I could have a future TAVR!  At that moment I was not interested in 10-15 years from then. I had truly been given a new lease on life! After all, I no longer had an ascending aortic aneurysm and I had a brand new healthy aortic valve! I felt indestructible! I believed with all of my being that my new valve would last at least 15 years +! A TAVR seemed like light years away! I didn’t even bother to ask what the acronym stood for!

What I didn’t understand was that data is beginning to reveal that a new tissue surgical aortic valve replacement has a 20% chance of failure within the first 5 years!

In the Valve-in-Valve International Data registry, 20% of patients had their surgical aortic valves replaced within the 5 years prior to the TAVR. It is a bell-shaped curve.  Some patients with transcatheter aortic valves degenerate earlier, and that is true also for those with the surgical valve -  Dr. Raj Makkar Medscape interview March 25, 2019.

2016 - The honeymoon is over so soon???

The fall of 2016 brought the beginning of the end to my honeymoon with my “new” aortic valve. My scan showed the first signs of calcification!

2017 – Just “lazy”!!

2017 revealed further immobility of the “new” valve.

I discounted my increasing fatigue, believing I was just “lazy”.

I was unable to clean my house and became short of breath when making my bed. I avoided steps whenever possible. I developed a rectal prolapse with complications requiring surgery and a subsequent surgical procedure to repair another rectocele.

 2018 – Denial can be dangerous!

2018 brought frequent episodes of angina requiring nitroglycerin, increasing SOB with simple activities, debilitating fatigue. I was in complete “DENIAL” still believing my “new” valve could not possibly be the problem. Dr. Raissi spoke to me by phone and said, “BJ, I am worried about you”. I said, “Don’t worry”. He quickly responded,” I do worry”! I finally heard his concern and boarded a plane.

Happiness after TAVR with my brave, loving husband and son!

Flying from the East Coast to LA, I began to have difficulty breathing. Once again I was in complete “DENIAL”! Once my son and I arrived in LA we walked from our hotel to the Tar Pit Museum in a heat wave! I became faint and needed assistance to sit to prevent me from falling! “DENIAL”! My breathing was so compromised that I required extra pillows during the CT scan to breathe. The CT revealed severe aortic stenosis.

Dr. Raissi called me following my CT scan, and said I should call go immediately to see a TAVR specialist, Dr. Raj Makkar, MD!

No more severe stenosis!
Sweet heart sounds after valve-in-valve TAVR

Disbelief, shock and denial continued in my head… I requested that I visit Dr. Raissi first. He firmly said, “BJ you need an immediate AVR and surgery is too risky with your co-morbitities. Go to Dr. Makkar’s office at Cedar Sinai.” Mercifully, Dr. Makkar was in town and not traveling, and I was given priority and worked into their extremely busy schedule.

07-11-2018 – From grieving over one valve to welcoming another

After extensive testing, insurance approval and TAVR education, reality finally started to sink into my being. To me it was no longer the loss of my “new” surgical valve. I began to sense how fortunate I was to have the stars align once again with a life-saving procedure.

 I was especially grateful for the Sentinel Cerebral Protection System that Dr. Raj Makkar utilizes to reduce stroke risk during a TAVR procedure. One study shows that Sentinel CPS captured visible embolic debris headed toward the brain in 99% of TAVR cases. Studies show more than a 60% reduction in TAVR-related strokes when Sentinel CPS was used noted by the “Society of NeuroInterventional Surgery” and “American Association of Neurological Surgeons”.

I can breathe!

07-13-2018 – After valve-in-valve TAVR “I can breathe….!”

7:00 a.m.-TAVR procedure with the expert hands of Dr. Raj Makkar and his amazing staff!

10:00 a.m.- I awoke to my family and Arlys Velebir by my side!

My first words were, “ I can breathe lying flat!”

07-14-2018 – Out of the hospital 30 hours later!

I was discharged approximately 30 hours from my TAVR once again feeling as if I had a new lease on life!!! I was at the right place at the right time and my stars aligned…truly a miracle!

09-08-2019 – BJ today - Very grateful!!

I become more GRATEFUL each day I have to live. I no longer focus on how long my valve will last or “what ifs”.

BJ (with Dr Makkar)
after TAVR
Happy heart, happy life!!!

As I continue to discover new ways to care for my body, I am finding improved health, vigor and contentment. I no longer eat processed foods and am limiting my carbohydrates which have resulted in a 35 pound weight loss. I was an insulin dependent and insulin resistant diabetic prior to making these changes. I no longer need insulin after 17 years!

I try to live each day by the words Dr. Makkar blessed me with upon my discharge. 

He was very encouraging …

He gifted me with these words….

"Go and Live!…Go to the Beach!…

Travel!…Have Fun!…Live your Life!...

Happy Life!…Happy Heart!!!”

From one BAVer/TAD to another:

Unlike me,

please  be prompt to share your symptoms

 with your trusted physician

 or confidant

 and

 resist the temptation of DENIAL….

May your stars align on your BAV or TAD journey,

 all the days of your life!

~ BJ Sanders

BAV Aortic Aneurysm Research Break Through

Diseased Aorta - Enlarged and Twisted Shape

This week we have exciting news to share about BAV aortic aneurysm research.

Desperate Need to Understand and Help
We are in desperate need to better detect, understand and appropriately help those with BAV and aortic aneurysms and their families.

Writing this, I remember  Doug Grieshop,  Forever 33, who left a young son and unborn daughter the day that his aorta ruptured. His BAV was never diagnosed, his aneurysm never found in life - only by autopsy.

Researchers Are Unmasking the Villains
At BAF we are very excited by published research, highlighted in the press this week, describing cellular villains in the aortic wall that destroy tissue around them and weaken the aortic wall.

This is work lead by Dr. Pickering, a research scientist, and Dr. Chu, an aortic and cardiac surgeon, who have collaborated to study aortic tissue removed from human patients. They have published their findings, reporting on the existence and trouble caused by abnormal smooth muscle cells in the wall of the aorta. 

According to Dr. Pickering, these abnormal cells produce an enzyme that eats away at the tissue around them, destroying and weakening the aorta. 

Following are press release and links with more information
Exchange Magazine  

Robart's Research

Dr. Pickering's background.

Dr. Chu's background.

Research Paper: Seno-destructive smooth muscle cells in the ascending aorta of patients with bicuspid aortic valve disease

Light Breaking Through the Mysteries 

Can Light Shed by Research Translate to Better Care?

This give us tremendous hope, but there is much more to do.

We know from the work done at Dr. Mona Nemer's Lab on GATA about the genetic abnormalities and BAV in mice families, who are so very much like our human families.

There are studies of abnormal blood flow through BAVs, of aortic wall stiffness.

There are studies about the leaflet configuration abnormalities of BAVs.

There are many things to study!

Now we know something important about the aorta tissue - that these abnormal smooth muscle cell secretions are breaking down the aortic wall!

There is not just one thing, but many things, to think about! What else is happening, that we do not know today? No doubt there is more to understand.

Light is just beginning to shine on multiple factors, some combination of which is making each BAVer experience somewhat unique, but with common tendencies and themes.

Aneurysms and Abnormal Smooth Muscle Cells
If we can identify these smooth muscle cell villains, can we get rid of them before they cause trouble?

Can we find out what causes these smooth muscle cells to become abnormal and prevent that from happening?

The Challenge of BAV Misunderstandings
It is a very difficult challenge when someone is the picture of glowing health on the outside, vibrant and active, but with a serious vulnerability on the inside - prone to aortic valve failure, aneurysm, infections.

Anything that sheds light on what is wrong on the inside helps both patients and physicians believe the reality of the BAV experience.

In the future, if aortic aneurysm disease were better understood, it might help physicians think about and look for BAV and an aneurysm in someone young and healthy-appearing with symptoms, like Doug Greishop, in time to save them. He lived in a state with world-renowned aortic surgical capability. It is tragic that he did not know, that no one knew in time, that he needed that expertise.

While we rejoice over the research and wait for it to translate into better care, we are very grateful for every successful aortic surgery that saves someone born with BAV and their TAV family members! And we do not forget those we have lost.

Today, we see light breaking through, but BAVers remain vulnerable. 

Someday, there will be more light than shadows in the experience of BAV families!

Answers are beginning to come,

Creating a Climate of Hope!

~ Arlys Velebir

                           Bicuspid Aortic Foundation