Bicuspid Aortic Foundation - The View from Here

Bicuspid Aortic Valve Families - Strangers in Two Worlds?

2012-02-20T21:42:00.000-08:00

Strangers in Two Worlds, Possibly Three ?

There are at least two worlds where someone
from a bicuspid aortic valve family

may find themselves.

Perhaps because of the sometimes subtle nature
of the abnormalities in these families,

those affected do not fit well in either of them.

Strangers in the World of Congenital Heart Disease?

There is a world for those with congenital heart conditions. It is defined by many complex problems that require life-saving surgery at an early age. These are hearts with problems so severe that without early intervention there is no hope. An example is Tetralogy of Fallot, which is actually four different abnormalities of the heart that are found together. Much of the pioneering heart surgery that began over 50 years ago was performed to help these infants and children. Surgical advancements have saved many of them, allowing them to reach adulthood. Their survival has resulted in an emerging, much-needed specialty for them as adults with congenital heart disease.

In contrast with these complex cardiac conditions, the bicuspid aortic valve must appear such a simple thing. Although it is the most common congenital heart defect, it may not cause any problems in childhood. Stenosis, or narrowing, due to unusual tissue, may force intervention in the young. But generally, the BAV does not find a home here. When I attended a large conference a few years ago, the congenital heart disease agenda held nothing for me to attend - the bicuspid aortic valve was not listed at all.

Strangers in the world of valve disease?

There is another world, the world of heart valve disease. This is a world focused on problems with the valves of the heart - most often the aortic valve and the mitral valve. The failing BAV is found here, included along with the other reasons for heart valve issues. Many with BAV, like my husband, are first treated in this world, undergoing life-saving valve surgery.

But the full picture is not contained in the aortic valve with only two leaflets. Dr. M. E. Abbott, through her work including publications in 1928 and 1936, informs us that bicuspid aortic valves are associated with aortic aneurysms and with coarctation. Dr. Abbot also documented death of those with BAV due to brain aneurysm.

Individuals and families notice that some of them have abnormalities expressed elsewhere in their body. And in some, there are issues such as blood pressure that surgery alone does not solve. Yes, many aspects of BAV make it a stranger in the world of isolated valvular heart disease also.

Strangers in the World of Brain Aneurysms too?

BAV families may be found in this world too. Is anyone expecting them here? I found it was all too true as I sat beside Carrie Mettler in Dr. Wouter Schievink's office, listening as he told her there was an aneurysm behind her left eye.

In May, 2010, "Screening for intracranial aneurysms in patients with bicuspid aortic valve" was published. This is the most recent but certainly not the first time that this finding was reported. While at Mayo in Rochester, Minnesota, Schievink and Mokri wrote about BAV families with arterial dissections in 1995 - "Familial aorto-cervicocephalic arterial dissections and bicuspid aortic valve". However, in her comprehensive work on congenital heart disease, Abbott reported in 1928 on BAV, coarctation, and brain aneurysm. Abbott documented four BAV cases of Sir William Osler in the Atlas of Congenital Cardiac Disease, 1936, Plate IX. In Figure 7c, one of Osler's patients is described as a 20 year old man who presented with symptoms of endocarditis, but who died suddenly of a ruptured aneurysm in his brain. Dr. Abbott's comment was "(?congenital)". In publishing her work, Abbott meticulously lists physician references back into the 1800's, documenting death due to brain aneurysm, while noting the condition of the BAV and aorta.

Who will take up this challenge today,

so that more BAV individuals and families
will not be strangers, unrecognized, in this world?

Families with BAV need a world focused on them;
a place where the variation and complexity of this condition are acknowledged.

It was when my husband's aortic aneurysm was discovered in 2001 that I began to understand there was much more to the BAV experience. We were shocked by the diagnosis of the aneurysm. I remember very clearly my husband's surgical consultation at that time. Would this surgeon understand? Yes, he not only understood the connection between aneurysm and BAV, but the blood pressure issues as well. Finding understanding and help in that surgeon's office was an unforgettable moment. That day, we found a place where the history of a bicuspid aortic valve was not trivialized. We found a place where my husband's plight was not an unexpected stranger.

Some years later, I listened on the phone as my husband spoke with another family member, just diagnosed with a BAV. A sports physical had found the abnormal, fish-mouth aortic valve. It was then that we began to understand that what my husband had was not isolated to just him. Having seen my husband go through two surgeries by this time, no one in the family was under illusions about what having "it" might mean.

Later still, my husband's sister began to have "heart" problems. What did she have, two leaflets or three? Even with the best imaging, there was some question. Her valve had definitely calcified and needed replacement. Surgical removal dispelled all doubt - her aortic valve, calcified and stenotic, had three fully separate leaflets.
Locally, despite her family history, she was initially viewed as just an isolated case of trileaflet aortic valve stenosis. But that was not the case. She had something else, an ascending aortic aneurysm. We began to understand that our family is like the thirteen families in a paper published in 2007 - "Familial thoracic aortic dilation and bicommissural aortic valve: A prospective analysis of natural history and inheritance".

It can be dangerous to find yourself in a world of isolated valve disease if you are from a BAV family. In that world, the diseased aorta that can tear and rupture may not be recognized and checked if the aortic valve has all three leaflets. We learned that this is the bottom line - when someone has a BAV, others in their family may be at risk of aortic aneurysm, dissection, and rupture, even though their aortic valve has three normal-appearing leaflets.

Strangers No More?

The goal is that no one with BAV in themselves or their family should find themselves a stranger. They should not doubt the reality of their own experience because they do not fit common perceptions about BAV. They should not suffer injury or death prematurely in a day when there are more resources to help them than ever before. Rather, they should have access to all the life-saving capabilities available today.

It is encouraging to see more accommodation, more recognition of the needs of BAV families. Northwestern is hosting a webcast tomorrow, entitled "Specialized Care for Patients and Family Members with Bicuspid Aortic Valve Disease". And new programs are springing up - those who find their way to Valley Heart in northern New Jersey will find a specialized TABAV Program - they will not be strangers there.

Tonight, I gently turn the pages of a book, yellowed and musty - the American Heart Journal of 1928. It contains Dr. Abbott's paper. I think of the Webinar from Chicago tomorrow, the various programs springing up like the one at Valley Heart, where the totality of BAV in families is not a stranger.

I believe Dr. Abbott would be pleased.

Arlys Velebir
Bicuspid Aortic Foundation

A Heart for the Race - Skiing a Marathon

2012-02-19T23:44:00.000-08:00

Some of the OSCR participants

Seeley Lake, nestled high in the mountains of western Montana, is known for its beauty. The ski trails of this community were the setting for the 30th OSCR (Over Seeley's Creeks and Ridges) Ski Race on January 29, 2012. Nordic (cross country) skiing is well known as a vigorous cardiovascular workout , and this skiing marathon was no exception.

Bob Gies (right) skiing the OSCR 10K, January 29, 2012

Everyone there was outfitted with their ski gear, but among the 10K participants was one 67 year old man with some very special equipment inside his chest. This man had a special heart for the race!

Athletic all his life, Bob Gies underwent major surgery in 2010, addressing three distinct problems with his heart and aorta. The seeds of those problems were present the day Bob was born. He had come into the world with an aortic valve that had only two leaflets. By the time he had surgery, his bicuspid aortic valve was calcified, his ascending aorta had ballooned into an aneurysm, and the "electrical" rhythm of his heart needed some help too!

In a prior blog, Bob's experience before and after surgery is given in more detail.

A heart for the race and a smile that says it all!

Bob went through cardiac rehab following his surgery and found the monitoring reassuring as he gradually healed and recovered his stamina. Sometime in 2011, he decided to enter this race, a true cardiovascular challenge.

As Bob approached the finish line, do you wonder what he was thinking? Many things, perhaps. Awe for the amazing things inside his chest - a man-made, tissue aortic valve faithfully opening and closing with every heart beat, the Dacron graft taking the pressure of the blood gushing out of his heart, and the "remodeled" electrical pathways - all the things that surgery had given him.

Finish Line!

Bob carried something else inside his heart as he powered through those trails on his skis - tremendous gratitude for the skilled hands of his surgeon, who gave his heart, his life, back to him again.

Yes, Bob had the heart for this race! He finished!

Tsunami of Hearts - Bicuspid Aortic Valves and Heart Month 2012

2012-02-12T16:23:00.000-08:00

BAV and Heart Month

Doug Grieshop at 2 months,
born with undiagnosed BAV,
died of aortic rupture at age 33

Somewhere today, a child is born with a bicuspid aortic valve. Along with the cries of the newborn, is there another sound? Will a mumur catch the doctor's ear? Sometimes perhaps, but the tiny valve with only two leaflets may make no sound at all.

What does that little two leaflet aortic valve

portend in the future

for this beautiful child, born today?

A Very Old Problem
First drawn by Leonardo da Vinci and capturing the attention of many great physicians in the pre-surgical era (Osler, Abbott, Lewis, Grant, Bishop, Trubeck(3) ), the bicuspid aortic valve is not a new discovery. Interestingly, today's statistics for congenital heart disease often do not include it, typically counting only those heart deformities requiring intervention in childhood.(1)

A True Tsunami - Millions of Hearts
The magnitude of the impact of BAV is tremendous, injuring or killing more individuals than all other heart defects combined.(2) The reason is that there are so many BAVs in the world. Actual statistics are limited (2), but using an estimated 2% of the population, in the United States alone, the number with BAV in the United States would be just over 6 million. How many of them will at some point be injured or die?

Seemingly Simple, Well Known, Still Not Understood
"We don't know very much about bicuspid aortic valves." These were the words of a physician, quoted by a shocked, grieving young widow. A physician who went to the service for her husband, who sat with her and reviewed all the reports - both of them trying desperately to understand what happened. Why she came downstairs and found her husband dead. It can seem safe to watch and wait, and then there is a sudden death that is not from aortic dissection or rupture. In the files of the Foundation is an autopsy report, along with a beautiful picture of another young man and his daughter. Sudden death, nothing found except a bicuspid aortic valve. Not as common perhaps, but still part of the story, along with aortic stenosis, aortic regurgitation, endocarditis, brain aneurysm, aortic aneurysm, aortic rupture, aortic dissection.

Dr. Abbott's Work
In 2011, the Foundation highlighted the work of Dr. Maude E. Abbott. Individuals and families affected by BAV are needed to change the future for the child born today with BAV - to move forward from where we are, to help find answers to the many questions that remain about BAV.

Be a "Heart Donor" in 2012
Contact the Bicuspid Aortic Foundation at contactus@bicuspidfoundation.com if you would like to help. And please consider a donation to support the search for answers. For as little as $10, you too can be a "heart donor" for the child born today with a bicuspid aortic valve.

References
(1) The Incidence of Congenital Heart Disease, Hoffman and Kaplan, Journal of American College of Cardiology, Volume 39, I ce ssue 12, June 19, 2002.
(2) Clinical significance of the bicuspid aortic valve, C Ward,

Heart

2000;83:81-85 doi:10.1136/heart.83.1.81
(3) Atlas of Congenital Heart Disease, M.E. Abbott, 1936, Plate IX.

"Special Forces" at War with Thoracic Aortic Aneurysm, Dissection, Rupture

2012-02-04T20:22:00.000-08:00

Special Forces
I recently discovered a warrior hero in my ancestry. Not one of the ancient Vikings from whom we are descended, but a modern day hero. He was among a very select special operations group in World War II, the Alamo Scouts, and later, unable to resist rejoining the fight, became a Green Beret.

I am so sorry I never met him. He died in 2006. I am getting acquainted with him through a book called Raider, as well as video and articles about him. Galen Kittleson is honored for going right into the thick of the enemy and freeing their captives, going on more raids to free prisoners of war than any other soldier. Through him, I am learning more about the unique role elite forces perform, keeping all Americans free and safe.

It has reminded me of the fight against aortic disease.

Drawn to exploring this subject further and listening to video of Marcus Luttrell, who wrote the book Lone Survivor, I was again struck by parallels to the fight with an enemy who is elusive, deceptive, ruthless, deadly - the fight with thoracic aortic disease.

The Enemy
Aneurysm - it is an old word, tarnished by death and devastation. In lay terms, it is spoken of as a time bomb, a balloon that can burst, wreaking havoc inside and causing sudden death.

Dissection (tearing but not complete rupture) - this less familiar word has entered the public square more recently, again associated too often with deadly consequences.

These two are the most familiar hallmarks of the enemy, aortic disease in the chest, whose presence has been under estimated, easily confused with coronary artery disease.

The Victims
Who does the enemy target? Today we know there are categories of people at risk, including the millions like my husband born with bicuspid aortic valves. But it is important to remember that this is an enemy that can cause trouble across a wide range of humanity at all ages. One evening a few of us met in a restaurant to discuss organizing the Bicuspid Aortic Foundation. Just before that meeting, I visited two people in the hospital. Both had an incision line down the middle of their chests. Both had Dacron inside. One was a little boy about two years old. The other was a man aged 52. One had Marfan Syndrome, one was born with a bicuspid aortic valve, but threatening them both had been the common enemy, an aortic aneurysm in their chests.

You might think that, unlike soldiers, the physicians called to do battle are safe. No, they have no special protection. They too may be in danger. ER physicians are on the front lines when many diseases attack, including this one. As I write, I am thinking of an ER doctor who died suddenly, presumed to be the victim of a heart attack. There was no autopsy. Later an aneurysm was found in his brother's chest. What really killed that ER doctor? Dr. Michael DeBakey, who pioneered aortic surgery and the use of Dacron to replace diseased aortic tissue, was himself a victim when his aorta dissected in 2005. Surgery was performed, placing the Dacron he had discovered into his own chest.

Exactly how many dead do we bury because

of an enemy called thoracic aortic disease?

No one knows.

Statistics are incomplete. Without an autopsy, even in death the enemy may hide. The heart, completely innocent and sometimes crushed in a pool of blood, is blamed instead. In a family who lost three brothers, it was only with the third sudden death that the enemy was unmasked, putting other family members on guard. Three similar sudden deaths, only one counted against aortic disease.

The Fight
Wars have been declared on heart disease and cancer. At the same time, battles with aortic disease in the chest went on more quietly, mostly out of the public eye. Surgeons began to win more battles, especially when the enemy was found before doing any damage. But surgeons can not fight an enemy that is not found.

Fighting thoracic aortic disease has many parallels to guerrilla warfare, hand to hand combat. The enemy may lurk silently, and if exposed, mimic something else quite harmless. Unless someone recognizes it is not anxiety, not indigestion, not asthma, and keeps looking, keeps thinking . . . the enemy remains hidden. When chest pain or pressure threaten to expose it, the enemy may distract by pointing to the heart - but there is no heart attack. There is no pulmonary embolism either. What else can suddenly launch a deadly strike in the chest ? The enemy is a diseased aorta, too often never even on the radar screen.

Those who fight such an enemy need special skills, honed by thinking and studying the enemy's tactics. As Marcus Luttrell has said, the fight does not come with rules clearly written in black and white. Who is up to such a fight?

Never Quit, Never Surrender
From the 1940's Alamo Scouts to the present, some things in particular stand out about these elite forces. They know the enemy. They never quit. They never think they are going to lose. They trust each other completely. They fight to the death.

If you had thoracic aortic disease,

who would you like to fight the battle with you?

I remember clearly a conversation between two doctors about a patient. One said it was just too much of a mess, too much damage had been done inside. I don't remember what the other doctor said, but I remember what he did. He kept fighting. He won. Who do you want on your side?

My husband had already been in some battles - first replacement of his bicuspid aortic valve and then removal of an ascending aortic aneurysm - and so, I dared to hope the fighting might be over. And then one night I found him, completely helpless, collapsed on the floor. As the paramedics wheeled him out the door, who do you think I called? Someone who would say it was too much of a mess, that it was too hard, there was too much damage? Someone who would say we would never know what happened, never find who the enemy was? No, I called someone who will always fight. Someone who will never give up. Someone who never thinks the battle is lost. And that is why I still have my husband today. For some like my husband, there are lulls between battles, but we can never let down our guard.

Volunteers
Members of these elite military fighting teams are volunteers. Of those who volunteer, very few qualify. Recently I spoke with someone who has volunteered for the aortic disease fight - someone who cares about those born with bicuspid aortic valves and all others in danger from aortic disease in the chest. Someone who is studying the tactics of the enemy. It gave me fresh hope, fresh courage for the battle. There are still volunteers.

If I could speak to Galen Kittleson today, I would thank him for his service to our country. And as I imagine our visit, we would speak about our common ancestry. But at some point I would tell him about this fight. I know he would understand the cause. He would understand the horror - the dead, the injured, the devastated families. He would understand the victories too.

But one thing he would never understand - giving up the fight.

May there be special volunteers to take this fight to those in danger - to unmask and disarm the enemy among us, thoracic aortic disease. And if you have this enemy within you, may one of them come to your rescue.

Arlys Velebir
Bicuspid Aortic Foundation

Life from Death - The Contributions of Patients

2012-01-08T16:43:00.000-08:00

There are two people that are very special to me. I know very little about them, not even their names. They made great contributions to medicine and surgery. No, they were not physicians. They were patients. They both lost their lives, and they both contributed to the care my husband would one day receive.

Death from a Torn Aorta after Valve Surgery
The first man was born with a bicuspid aortic valve. I believe it was sometime in the 1980's that he needed surgery to replace his aortic valve. He came through the surgery well and was moved from the operating room to intensive care. But sometime during that first night, things went wrong. By the time it was realized that his aorta had torn (dissected), it was too late - they could not save him. When I think of him, I only hope that lying in that hospital bed, probably still on a respirator and unable to speak, the drugs he was given for pain kept him from suffering and that he slipped away in peace.

A dissected aorta after valve replacement? This was not supposed to happen. Why did his aorta tear? There was a young surgeon at that hospital, still a resident. From that day on, this surgeon never forgot what happened to this man with a bicuspid aortic valve. He never forgot that those with bicuspid aortic valves may have abnormal, delicate aortic tissue.

Blood Pressure and Aneurysms
The second man was about 50 years old. He was an airline pilot, and like my husband, he had an ascending aortic aneurysm. He needed surgery and was sent to a surgeon for consultation. A surgery date was set, and the pilot would follow up with his other physicians regarding medical care and additional pre-surgical testing. Before his surgery date arrived, this man's aneurysm either tore or fully ruptured. He died.

From that moment onward, the surgeon vowed that he would personally manage the blood pressure of all his aneurysm patients prior to surgery. This video explains why.

In simple terms, it is similar to the balloons that I loved as a child - as I blew air into them, a point came when the pressure and size were too great for the wall to hold, and my beautiful balloons would burst.

A "Balloon" in my Husband's Chest
When my husband's aneurysm was diagnosed in 2001, we had no idea where to turn. The aortic aneurysm in his chest, just above his artificial aortic valve, had been "discovered" - it was not looked for intentionally. We confronted a vital question immediately - where could we find someone that could help him?

Prior to this, having been born with a bicuspid aortic valve had been spoken about in very optimistic terms. That rosy view had lost its credibility with me. No one had linked the fact that he was born with a bicuspid aortic valve to a risk for developing an aortic aneurysm, although having read Dr. Abbott's work from the early 1900's, I know she clearly identified this risk.

Knowing he had a bulging aorta in his chest was one thing. Knowing that he had high blood pressure that was not being treated added to our desperation. (I just couldn't escape those childhood memories of my balloons abruptly bursting.)

Meeting an Aortic Surgeon
We walked into an office for a consultation with an aortic surgeon. On our own, without a referral.

Along with many questions I had, there were two major things I was looking for as we entered the surgeon's office: someone who appreciated the connection between a bicuspid aortic valve and aneurysm of my husband's aorta, and someone who would be concerned about my husband's blood pressure.

I braced myself for the response when we mentioned that my husband was born with a bicuspid aortic valve. Would there just be a "that's no big deal" attitude? And what about that high blood pressure?

Perhaps now is the time to tell you that, unknown to us, we had walked into the office of an aortic surgeon who as a young resident saw a man die from a torn/ruptured aorta after valve surgery. This was also the surgeon who was never able to help the airline pilot, because he died too soon. A surgeon who has never lost another aneurysm patient prior to their chosen surgery date, because he personally monitors their blood pressure and adjusts their medication.

Two men who died, and the surgeon who vowed that never again must such things happen - because of them, my husband received immediate, expert help with his blood pressure and the surgery he needed.

Becoming A Well-Informed Patient
It remains a tragedy that those two men lost their lives. I share their stories here, with tears in my eyes. I think they would want you to know and to avoid what happened to them. Tragedy is greatly compounded when we fail to learn from it, and stop it from being repeated. As patients, we can learn what is vital for our treatment and seek it out.

There have been many who have experienced aortic catastrophe. May our tears pay tribute in some small way to all who have gone before us. In recognition of them, we can become well-informed patients - seeking information, asking questions, and making informed decisions with the knowledge we have gained.

To that end, in 2012 the Bicuspid Aortic Foundation is launching an informational program called "The Well-Informed Patient". It is intended to save many lives. Our goal is that no one should die from treatable conditions.

May life come from life, not death, as we share our lives together, creating a climate of hope.

Arlys Velebir
Bicuspid Aortic Foundation

In Memory of Sahib Singh Vaseer: Endocarditis and Bicuspid Aortic Valves

2011-12-18T21:51:00.000-08:00

Sahib Singh Vaseer
July 7, 1995 - March 23, 2006

On the Bicuspid Aortic Foundation's website is the heartbreaking story of a little boy, Sahib Singh Vaseer, whose life was taken by infection just over five years ago. No one knew that Sahib was born with a bicuspid aortic valve, increasing his risk of infection attacking his heart.

In the words of his family, " Our lives were shattered and we were devastated. The earth had moved from beneath our feet.... How could such an active boy go away like this?"

At this time of holidays and family gatherings, we are painfully aware of those who are missing - taken from their loved ones by various complications of Thoracic Aortic Disease. We need to be reminded that infection is a serious threat and remains a very real risk to those born with bicuspid aortic valves.

Following is a video clip about the risk of infection, taken from the Bicuspid Aortic Foundation's 2011 Conference. We share it here in loving memory of a Sahib.

Aortic Valve Stenosis, the Risk of Under-Treatment, and a "Boot-Shaped Heart"

2011-12-18T17:42:00.000-08:00

Some hospitals have beautiful natural settings, and we recently found ourselves at one of them - high up on a hillside overlooking the sparkling, blue Pacific Ocean. We were there recently for an echo, and I remembered again the first time I came there, with my very sick husband. The beauty of that setting was rather wasted on us then.

Aortic Stenosis and Heart Failure
My husband had a life-long heart murmur, but never had the "classic" warning symptoms of valve disease - shortness of breath, fatigue, swollen ankles due to fluid retention. Somehow his body compensated so well, that no one knew there was just enough fluid gathering in his lungs to make an inviting place for bacteria to grow. I was terrified as the fever spiked and his body was wracked with chills. It was off to the ER - abruptly he was in full blown double pneumonia and then obvious heart failure.

The pneumonia was very serious - I remember being told there was only one other drug choice if the antibiotic didn't work - it did. I understood how deadly pneumonia can be even in younger people then. Jim Henson, creator of the Muppets, died of pneumonia later that year.

We began a very personal crash course on aortic valve disease. I remember his Internist drawing a picture of a heart and showing us where all the valves were. We learned it was his aortic valve that had a problem - the valve that had made just a little murmur when he was born had gradually become more noisy and now made a horrendous rumbling, even to my untrained ear without a stethoscope.

We learned it was aortic stenosis - calcification had built up on the two leaflets he was born with until now the valve opening was only 0.5 cm2 - classified as critical.

Over the years, his heart muscle had been very strong and pushed the blood out to his body through that bicuspid aortic valve as gradually it narrowed. Working so hard, his left ventricular muscle thickened and became misshapen - they told us his heart was shaped like a boot.

Aortic Stenosis Can Be Deadly
In the video below, from the Foundation's 2011 Conference, statistics are presented about aortic stenosis, and how quickly death follows once it becomes severe - half of those with untreated severe aortic stenosis die within 2 years of their diagnosis. Given how suddenly and severely a crisis developed in my husband, I know he did not have even that much time.

Under-Treatment of Aortic Stenosis
The magnitude of the aortic stenosis problem is shocking: 300,000 Americans that need treatment today, but only 80,000 surgeries done annually. Why? It is important for individuals and families to know that aortic stenosis can be under-treated. There is no medical treatment, and if timely evaluation and discussion of surgery does not happen, the patient may become too sick. Percutaneous valve insertion (TAVI) has just been approved in the US for those too sick to have surgery - but that approval does not include those with BAVs. This video clip from the Foundation's 2011 Conference highlights the concern about under treatment and the possibility for excellent outcomes even at older ages.

Be Well Informed and Avoid Under-Treatment
Aortic stenosis is a very serious condition - being well informed and finding expert care is important. Taking advantage of the information resources available today can make such a difference. It is possible for individuals to consult with surgeons directly, obtaining multiple opinions as needed to understand their options, in order to avoid under-treatment. At the same time, the aorta should be evaluated for enlargement in those who have BAV or come from a BAV family.

My husband's heart murmur was never really "followed" most of his life. He seemed well, and we just did not know any better. As I walked across from the parking lot into the hospital, I remembered how little we knew, how vulnerable we were those many years ago. He is among those fortunate ones whose hearts "remodel" and come out of failure. He fully recovered after his failed BAV was replaced. But that does not always happen, leaving otherwise healthy people impaired by a damaged heart. In order to receive the full benefit of aortic valve replacement or insertion, the heart itself should not have been injured by the effects of aortic stenosis.

May all those with aortic stenosis today receive the information and help they need to keep them safe, and have well-timed intervention to keep widely open that vital flow of blood from their heart.

Best wishes,

Arlys Velebir

Bicuspid Aortic Foundation

One Day In December: A Window into the World of Thoracic Aortic Aneurysm and Dissection (TAAD)

2011-11-30T21:05:00.000-08:00

Aorta has the
shape of a
candy cane

Disclaimer: This account describes what happened to three individuals with Thoracic Aortic Disease (TAD) on the same day in December 2004. The TAD details are factual, but the personal details and timeline are altered to protect privacy. Any similarity to actual individuals and families is unintended.

Aortic valves in the heart
are often
involved in TAD

This is what happened on one day in December to three men, close in age, and their families. They did not know each other, and they did not know they had something in common. Something called Thoracic Aortic Disease (TAD).

5:30 AM
In a large medical center in a southern state, it is 5:30 AM. This is the day, the hour, the moment so carefully planned. It is time for a fireman, accustomed to protecting and saving others, to place his own life in the hands of a highly-skilled surgical team. Today, they will remove the aneurysm in his chest. Today, they will save his life.

In this same southern city, another man and his wife will soon be awake and begin their day. He will leave for the office, and a little later she too will go to work. It looks like just an ordinary day ahead.

In the suburbs of a city in a northern state, it is also 5:30 AM. A man, his wife, and their children are all still sleeping. This man stayed home from work yesterday. He has never missed worked because of sickness before. He had a vague discomfort in his chest, enough to keep him home but not enough to really alarm him. He spent a restless night, but is sleeping now.

8:00 AM
The fireman is in the operating room, and surgery has begun. The surgeon will soon be removing the aneurysm in his ascending aorta. How did they find this aneurysm? This man, an active outdoorsman and athlete, had never really been sick in his life. But one day he felt something strange in his chest. No pain, just pressure. This feeling of pressure was there day after day. It did not go away, so he found himself in the doctor's office. They checked him out and found the aneurysm. Two different physicians said it wasn't big enough for surgery yet, and he was told they would watch it. But this man saw his own father die suddenly. His Dad never made it to the hospital, and he wonders now if that sudden death could have been a ruptured aneurysm, not the heart attack it was assumed to be. Not satisfied, he sought out an aortic surgeon, who told him that if, even with blood pressure medicine, the pressure remained in his chest, it would be time for surgery. The pressure feeling did not go away, and now he is in surgery. His aortic valve will also be replaced.

Far to the north, the family has now started their day. Because her husband still doesn't feel well, his wife has just told him he must see a doctor today. He agrees, saying that he will be ready to go in a little while. Then, he doubles over in severe pain and at this moment is being rushed to the local emergency room, just a few minutes away.

Returning to our southern city, the third man has just started his workday, reading email at his desk. He is feeling well and looking forward to the day. When he awoke this morning, he remembered how fortunate he was to be alive and well. Nine years ago, he had surgery on his heart. They replaced his aortic valve, giving him a mechanical one in its place. The valve has worked perfectly. It has been nine good years he would not have had without that artificial valve, softly ticking like a watch in his chest. He and his wife are grateful to have their health and each other.

This is how this day in December started for three men. All three of them are under 50 years old. All three of them have an aneurysm in their chest, but only one knew about it as they started their day.

At the end of this day, what has happened to each of them?

5:00 PM
The fireman is in intensive care now. His family has seen him, and they are now in the waiting area. He is starting to come out of the anesthesia, and everything is looking good. His family will soon be allowed to briefly see him again. The surgery was over about 2:00 pm. The surgeon told the family that the aneurysm had grown in just the last month, and the tissue was very thin. This man's aorta may not have held together much longer. That feeling in his chest was an important messenger. Surgery was a necessity, and today was the right day. This family has an additional reason for the stress they feel. When the surgeon spoke to them, he looked at this man's younger sister and told her that she needs to be checked too. They understand now there could be other family members who also have TAD.

In the same waiting room, there is another family. This is the wife and parents of the man who began his day at his desk, reading his email. They never expected to be here today, waiting while the doctors try to save their loved one. His wife is in shock. She remembers how well her husband was when he left for work this morning. How could anything be wrong with his heart? Everything has been just fine since they replaced his valve nine years ago. They told her something about an aneurysm, somewhere in his heart. How could that be? She thought aneurysms were in the brain. She got a call this afternoon at work, saying that her husband collapsed at the office and was rushed to the hospital. She was told to come immediately. She has no idea what is happening. She thought her husband's problems were over when his valve was replaced nine years ago.

Many miles to the north, a woman and her children are back in their home, surrounded by family and friends. The man who left home in terrible pain this morning, their beloved husband and father, is not there. He will never be with them, never walk through their door again. He left them forever this morning, in the emergency room of their local hospital. Alive when he arrived there, after only a few minutes he slumped back and was gone. His children's last memory of him is the terrible pain he had as he left home. There was no time to say goodbye.

Three strong apparently healthy men, all affected by one life-threatening condition, Thoracic Aortic Disease.

Did the man still in emergency surgery survive? His family was told it was not looking good. If he did live, how much damage did the dissection of his aorta cause to his body? Some people do survive a life threatening tearing of the ascending aorta, but many do not.

The fireman recovered well and went back to work. His sister has been checked, and she too has an aneurysm. Other family members are being screened also.

The third family remains in deep grief. This husband and father was always so strong and healthy, and in a moment he was gone. His children, brothers, and sisters all are being scanned for aortic enlargement.

Three strong, seemingly healthy men, who all had ascending aortic aneurysms. Prior to that day in December, they all needed what only one of them received: a CT scan or an MRI of their aorta, followed by elective aortic surgery. An echocardiogram also might have found these aneurysms, if its images included the ascending aorta.

Every day, through out the United States and the entire world, variations of what happened to these men and their families continue to take place. Increased awareness of the aorta in the chest, proactive screening, and elective surgery are needed to prevent the tragedies caused by TAD.

Candy Canes and Hearts are symbolic of TAD

This December, your donation can help the Bicuspid Aortic Foundation make a difference in the lives of those with aortic valve and thoracic aortic disease. Please join us in saving lives by supporting our Candy Canes and Hearts Campaign! Donate to Candy Canes and Hearts here.

Aortic Dissection and Cardiac Tamponade

2011-11-27T21:44:00.000-08:00

One of the greatest threats to life associated with thoracic aortic disease is uncontrolled bleeding. The diseased aorta may tear (dissection) or rupture.

When blood escapes from the part of the aorta enclosed by the pericardial sack around the heart, it results in what is called cardiac tamponade. The following video shows how this happens. The example used is a tear that begins in the inner lining of the descending aorta and goes backward, against the flow of blood. Often the tear may start in the ascending aorta and continue forward, in the same direction as the flow of blood. In either case, if blood leaks out of the aorta into the pericardial sack, cardiac tamponade results.

Some of those with aortic dissection may survive for hours or even days. However, if the outermost layer of the aorta is breached at any time, blood flows out of the aorta. As seen in the video, it may fill the pericardium, resulting in death as the heart stops beating. If the leakage is from a section of the aorta outside the pericardium, blood is forced out into the body with each heart beat, also resulting in immediate death.

The Bicuspid Aortic Foundation hears from those who have lost a loved one in this truly horrific way. In sharing this video, we hope that it will increase understanding of what an aortic emergency truly is. This is why it is so important that aortic disease be found early, monitored carefully, and surgery take place before a crisis occurs. May we all help raise the visibility of BAV and other forms of TAD that put far too many lives at risk of sudden death.

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2011-11-24T09:47:00.000-08:00

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Bicuspid Aortic Foundation Annual Conference by Webinar, November 12, 2011

2011-10-30T13:41:00.000-07:00

Advances in surgery is
a popular topic at BAF meetings

This year, in lieu of an in person conference, the Bicuspid Aortic Foundation is hosting their annual Conference online. This format allows sharing leading-edge information about the treatment of BAV and TAD to a larger community without the need to travel.

The date is Saturday, November 12th.

The time: 11 AM, Pacific Standard Time

The topic: Aortic Valve Surgery

There will be no charge, but the number of spaces is limited.
To Register: It's easy! Click Here to Register!

Looking forward to having you join us!

Smog Checks and Heart Valves

2011-10-24T14:34:00.000-07:00

One sunny Saturday afternoon, I found myself chatting with another woman who also was waiting in line. We had a common goal - getting a smog test done on our cars. Somehow, our conversation turned to heart valves, and we discovered something else in common - our husbands both had valve replacement surgery just over 20 years ago. Their surgeries were done locally only a few months apart - the same hospital, surgeon, and type of mechanical valve. Her husband had two valves replaced - both his aortic and mitral. My husband had his bicuspid aortic valve replaced at that time.

As we chatted, she told me that her husband was still living, but he had suffered more than one stroke over the years. I told her that my husband suffered a massive stroke caused by strands of tissue that had formed on the intake of the prosthetic valve, broken off, and traveled to his brain.

She told me that his doctors want to replace her husband's mechanical valves. She was not specific about what was wrong, why the doctors wanted to "redo" them. I told her that my husband's mechanical valve was replaced in 2006, as soon as possible after his stroke, to prevent even further injury to his brain.

And then she voiced what we both were told two decades ago - that mechanical valves would last much longer than a human life span. Yes, they were supposed to last . . . . And it is true, the valves did not break, but still, here we were years later - despite the innovation and careful engineering of these valves, there had been complications, serious complications, after all.

In addition to the tissue strands that had formed on his mechanical valve, my husband also had something called pannus, a kind of scar tissue that his body had formed around the valve opening. There are many questions about why and how this happens - and no real answers. In my husband's case, a little over 15 years after its implantation, the valve that had kept him alive became a significant threat to him, and the affects of the injury to his brain from those strands remain with us nearly six years later.

Mechanical Valve With Pannus & Strands
Removed From My Husband in 2006

I realize now that the mechanical valve implanted in my husband had only been used in humans for 13 years at the time he received it. There was a great deal of understandable enthusiasm at that time about improvements over prior, more primitive mechanical solutions. But time has continued to inform the medical community about both mechanical and tissue valve options. Today, the message to heart valve replacement recipients is about choosing the valve they prefer. Yes, there is more information available about the outcomes, so that patients can make informed choices about repairing or replacing their heart valves. And there is part of that message that is important to understand - there is no perfect solution, but there are life-saving options. This recent paper from Japan , Current status of the mechanical valve and bioprosthesis in Japan, expresses well the message and decisions for those with heart valve disease.

Since that sunny afternoon, I have thought from time to time of this woman and her husband. She told me that he refused the offer to have surgery to replace his two mechanical valves. If he doesn't have surgery, I wonder what he will experience, how he will feel. And if he should at some point decide to have surgery, what the outcome would be.

I remember her face, her eyes, as we spoke - I saw something there beyond our words. We were two strangers sharing a rare understanding - the ups and downs, the joy and the pain that had come to us over the years because of heart valve disease.

Perhaps I am thinking of this today because I remember it is soon time for my husband's echocardiogram. We keep an eye on his "new" tissue replacement valve, as well as his own mitral valve, which is "a little leaky".

And I remember, that without something being done those many years ago, I would not have him here today. And I hope for better solutions, more understanding of the complexities, for those with heart valve disease.

At the Bicuspid Aortic Foundation, we do not forget that we must press for a better and brighter future for those with heart valve problems, including those who, like my husband, from their first breath of life face a future that will include heart valve disease.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

"We Thought That My Family Died of Heart Disease"

2011-10-11T21:03:00.000-07:00

Sadly, it is not unusual for the Foundation to hear from someone with thoracic aortic disease (TAD) who has already lost other family members - loved ones that they and everyone else assumed died from heart attacks. In one family, two brothers had already died of presumed heart attacks. When the third brother died, it was discovered his aorta had torn and killed him. All three men died in the prime of their lives.

One man wrote to tell us of the discovery of an aneurysm in his chest, and mentioned it was taken for granted that all the men in his family had heart trouble and died of supposed heart attacks. There were no autopsies. Could it actually have been their aorta?

The cause of these deaths is now questionable and deeply troubling, once a thoracic aortic aneurysm has been found in these families.

Michael Kirk is among those who in hind sight believe members of their families lost their lives to thoracic aortic disease. Michael, a cardiac intensive care nurse, spoke about his own experience with aortic dissection at the Foundation's Conference in 2007. There are some unfortunately familiar themes as Michael tells his story - "your heart is fine", "do you have anxiety?", "you don't fit the profile for someone with aortic dissection", . . . .

In the following video, Michael tells what happened to him. Incredibly, his aorta somehow held together for 68 hours before his dissection was diagnosed.

In the video below, Michael talks about how, despite being a medical professional himself, he was told "you have to be crazy to be here". When his dissection was ultimately found, it became apparent to everyone that they had not listened to him. It also became apparent that pre-conceived ideas about who has thoracic aortic disease and what they look like clouded the medical team's thinking and put Michael at grave risk.

A paper was published in September 2011 in Germany that addresses the importance of awareness: Diagnostic pathways and pitfalls in acute thoracic aortic dissection: Practical recommendations and an awareness campaign. The authors mention a poster campaign, "Thinking of it can save lives".

Finding TAD as early as possible is key to avoiding emergencies. But when someone with TAD does arrive at the ER seeking help, every moment counts in finding the problem. It begins with thinking about the aorta.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Thoracic Aortic Disease (TAD) - Looking For It and Finding It Early Saves Lives

2011-10-06T22:08:00.000-07:00

The Bicuspid Aortic Foundation issued a press release this week, Bicuspid Aortic Foundation Observes Thoracic Aortic Disease Awareness 2011, to bring attention to the risk factors associated with TAD and the importance of finding TAD earlier. Finding TAD early allows for proactive treatment in the most favorable setting - before a life-threatening emergency strikes.

On this Blog, the Foundation is featuring the experiences of some of those with various forms of TAD. In the video below, along with Dr. Sharo Raissi, Chairman of the Scientific Advisory Board, I speak about meeting some of those with TAD, and the great difference that information and finding TAD early can make.

Through out the remainder of 2011, the Foundation will continue to place special emphasis on finding Thoracic Aortic Disease as early as possible. Please help us by sharing this information with everyone you know.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

John & Tina Becerra - Life after Aortic Dissection

2011-09-16T22:26:00.000-07:00

Unlike Doug Grieshop, whose aorta completely ruptured and caused immediate death from massive internal bleeding, the inner layers of John Becerra's aortic wall tore, but the outer layer held together, making it possible for John to have surgery. In the video below, John and Tina speak about that experience and their life following John's aortic dissection.

May we all find our way to that place where every day is special.

Thank you, John and Tina, for sharing your experience with us.

Remembering Doug Grieshop - Thoracic Aortic Aneurysm and Dissection Awareness 2011

2011-09-10T12:26:00.000-07:00

In 2005, a young woman contacted the Foundation. Her name was Stacey Grieshop, and she shared with us the tragic loss of her husband in September the previous year - unknown to him, he had a bicuspid aortic valve. Also unknown, he had an aneurysm of his aorta in his chest. He died suddenly of aortic rupture. The physicians he had seen previously had not recognized what was wrong. An autopsy revealed what in life was not understood - BAV and ascending aortic aneurysm.

From the first, Stacey sought ways to tell Doug's story and save others. The Foundation declared September an awareness month beginning in 2005, and Stacey bravely shared that month of both joy and grief with everyone. Doug's birthday, their wedding anniversary, and the day of Doug's death are all in September.

All of us who volunteer with the Foundation are grateful to Stacey for continuing to share her story. This year, she spoke to a group of life insurance agents. Here is the video

Here is the Foundation's press release, issued in 2005 - September is TAAD Awareness Month. The words written then remain true today - there is life-saving treatment, but it must be detected in time. I regret to say that lives continue to be lost, wives and children left to mourn. There is still lack of awareness, as evidenced by this headline - Wrongful death suit filed after 21 year old dies of ruptured aorta.

In 2011, it is clear that there is still a great deal to do to prevent these tragedies. Thank you, Stacey, for all you do.

Bob Gies, BAV, Aneurysms, and the White Cliffs of the Missouri

2011-09-05T13:09:00.000-07:00

In September, Bob Gies always does something special on his birthday - he paddles down the Missouri River, past the amazing White Cliffs described by Lewis and Clark. This year, as he turns 67, this 40 mile canoe trip is extra special. The rock formations are a little more spectacular, Montana's big sky a little bluer, and the company of his wife and friends extra sweet as they paddle along and camp together. Why? Well, he missed doing this last year, for a very good reason. He was still recovering from surgery - major surgery that among other things removed an aneurysm in his chest.

A Young Athlete with a Heart Murmur

Bob has always been active, athletic. Even as he marks his 67th year, those many years younger can't keep up with him. But there was a period of time when Bob wasn't sure he would see this day. There were times in the past when Bob was afraid - afraid of something wrong in his chest, something he didn't fully understand.

Looking back, Bob remembers being told he had a heart murmur when he was just 14, during a physical before playing football his freshman year in high school. He was told he should be fine, and played football all four years. Bob always came through when they needed him. As a senior, he rushed over a mile. All these years later, everyone remembers that while others were good, Bob was an outstanding athlete in school. Bob still describes himself as always going all out in everything he does, pushing himself to the wall. After high school, he turned to hiking, biking, skiing, and fishing, which became lifelong activities.

Bob in June, 2011

After college, Bob and his wife lived briefly in Australia and New Zealand, but ultimately returned to their Montana roots, settling in the western part of the state, where the mountains provide the perfect setting for outdoor activities. He continued to be active into his 50's - pushing himself to the max and leaving those younger behind him. He remembers starting to feel tired as he approached 60 and chalked it up to just getting older. He found himself needing a nap and decided to sell his business. Then, in his early 60's, something happened that frightened him.

What Happened to Superman's Cape?
He was skiing with a friend on Lolo Pass when his heart rate jumped and pain in his chest traveled down both arms. In those moments on the mountain, Bob first seriously realized he might not be Superman anymore - something had cut off his cape. He returned to the car, took aspirin as a precaution, and when everything went away, chalked it up to indigestion. All was good until six months later when his heart began to race again. This time they had finished skiing, and Bob was driving down from the Pass. They switched drivers, and that time too, it went away.

Bob skiing again, the first winter after surgery

By the third time it happened, the snow was gone, and he was biking with his nephew. They were riding in the hills around town, nothing too steep, when his heart beat accelerated. This time, being nearby he decided to go to the hospital and be checked. At the onset his pulse was 220 - arriving at the hospital, it was still 196.

Hospitals and Doctors - A New Experience
Bob hadn't been in a hospital since he was born, and rarely had reason to see a doctor. As he stepped through the hospital door that day, that began to change. Ultimately he would seek several opinions, trying to understand what was happening in his chest. A whole battery of tests were performed. His heart murmur was brought up again, and he learned he had been born with a bicuspid aortic valve. The rapid heart beat episodes were given a name - "supraventricular tachycardia". With the tests and opinions, there were different solutions offered - an ablation for the rapid heart beat, surgery to bypass a blocked artery in his heart and replace his bicuspid aortic valve.

Somehow, Bob was not satisfied with what he was hearing. At times he felt pressured to do something, which only made him more skeptical. One doctor told him he could wait until he couldn't walk to his mailbox anymore to have surgery, not an appealing thought for an active man.

He kept researching his options, but life was difficult. Not knowing when his heart might begin to race, and the terrifying feeling when it did, made him very uneasy. When he visited his daughter in California, he would ask how far it was to the nearest ER. He began to feel like an emotional wreck.

In early 2010, he was desperately searching for answers and mentioned his BAV to a lifelong classmate and friend whose brother happened to be born with BAV - who pointed him to the Bicuspid Aortic Foundation. In March 2010, when Bob visited his daughter in southern California, he brought his test records and images with him, and a CT scan and office visit were arranged with Dr. Sharo Raissi. There it was confirmed that Bob did indeed have a BAV, but something else was explained that Bob had not been told despite multiple opinions. Bob's ascending aorta was bulging - he had an aneurysm in his chest. Fortunately, unlike many of those with BAV, Bob has low blood pressure, a good thing when there is weak aortic tissue.

Bob Chooses to Have Surgery
It was explained to Bob that in one surgery, three things would be addressed: his bicuspid aortic valve, his aneurysm, and his rapid heart beat. His BAV would be replaced with a bovine tissue valve, his ascending aorta would be replaced with Dacron under total circulatory arrest, and his rapid heart beat would be corrected with a Maze procedure. It was confirmed by both a cardiologist as well as the surgeon that there was nothing of concern in the arteries of his heart - no bypass was needed.

Bob returned to Montana and made plans for surgery. He wanted to recover during the summer and be well on his way to activity by fall. And that is what happened!

Back on his bike, September 2010

The Fear is Gone
Bob had surgery in May 2010, and recovered at his daughter's home. Returning to Montana, he spent the summer in cardiac rehab, while gradually increasing his activity. He learned to listen to his body, to pace himself as it healed. He was especially happy to hear how well his heart looked electrically. The rehab staff raved about the perfect tracings on his EKG. No more fast heart beats! By September and his birthday, he was biking again, but not yet ready for strenuous paddling down the Missouri. But he had a goal to aim for - seeing the White Cliffs in 2011!

Bob's first winter after surgery

By the time there was snow in the mountains, Bob was back to cross country skiing! In his first winter after surgery, he totaled 296.4 miles on skis, travelling between 4 and 12 miles in a trip.

Information Made All the Difference
Today, Bob credits the information he received from the Bicuspid Aortic Foundation as the first step in finding the solution he needed. He did need: replacement of his calcified BAV, removal of his ascending aneurysm, treatment for his tachycardia. He did not need bypass surgery (or stenting) of his coronary artery.

Bob fishing in 2011

The View from Here . . .

Perhaps if Bob had died suddenly on that mountain, in the absence of an autopsy it would have been ruled a heart attack. It would have been a great tragedy for us, because Bob is very close, like a brother, to our family. The classmate that pointed him to the Foundation is my husband's brother. It would have been crushingly devastating if I had learned too late that Bob had a heart murmur due to a BAV, but had never been screened for an aneurysm. I would have wondered if his aorta dissected or ruptured, and if his calcified BAV contributed to his sudden death. Today, we are all very grateful that is not our fate. Tragically, it continues to be the fate of too many with BAV and other forms of thoracic aortic disease.

The Montana skies were never more beautiful, the cliffs more enchanting, than when Bob floated down the Missouri this September, past the beauty described by Lewis and Clark so long ago.

Bob, you are an inspiration to us all.

Arlys Velebir

President and Chairman

Bicuspid Aortic Foundation

Richard Holbrooke and Thoracic Aortic Disease Awareness

2011-08-27T23:28:00.000-07:00

www.state.gov

I followed news reports of Richard Holbrooke's hospitalization closely last December. The valiant fight began on Friday, the 10th, and unfolded over the week end - two surgeries, the first an incredible 20 hours long, no doubt accompanied by state-of-the-art medical treatment. It ended in tragedy. He died on Monday, December 13th.

I remembered years earlier standing at a bedside in cardiac ICU, holding the hand of a survivor of an aortic dissection. I knew he had come close to death. His name was Richard too.

And I thought, as I have many times, of how important it is to detect aortic disease earlier, before it becomes life threatening. It is not always so difficult, as is sometimes implied, to find it before an emergency happens. True, not everything is known. But with what is known today, if risk factors and warning signs are heeded, technology can be used to find aortic disease in the chest. The information on this center's website, Risk Stratification: Is My Aneurysm at Risk of Rupture , especially number 2 under risk assessment, describes the implications of milder symptoms in those with aneurysms.

I wondered if there were any warning signs before the crisis in this case. So I searched online for medical clues about Richard Holbrooke prior to December 10th, and this is what I found from April of that same year:

From Reuters, U.S. envoy Richard Holbrooke to have heart treatment .

From The Voice in the Ear Stent Blog, by Burt Cohen, an article explaining Richard Holbrooke's angriogram. It mentions that no significant problem was found with the arteries of his heart, and he was cleared for overseas travel.

From Politico.com, Holbrooke cleared to travel . It ends with the words "good news on the Holbrooke health front." It was good news on the arteries of his heart - but that is not the only reason there can be something wrong in the chest. I found no public record of any further searching for a problem.

Just 8 months later, on Friday, December 10th, Richard Holbrooke, while at work at the State Department, became ill. He was, in fact, suddenly in very serious trouble.

From ABC, Ambassador Richard Holbrooke Hospitalized . This article mentions being treated by medical personnel at the State Department before going to George Washington University Hospital for a blood clot. Blood clot in the lung (pulmonary embolism) is one reason for symptoms in the chest. There is no mention of any thought of his aorta at this point.

At the hospital the tearing of his aorta was found, and many articles describe what followed - the first long surgery, his critical condition, another surgery, and his death on Monday, December 13th.

I wish I could write about a different outcome for Richard Holbrooke - that finding the arteries of his heart were normal, tests were then scheduled to look at his aorta. Testing revealed an aneurysm, his blood pressure was strictly controlled with medicine, and he was scheduled for aortic surgery. Out of the hospital in a few days, recovering over perhaps a few months, and then a return to full activity. This is the outcome that others have been fortunate to have. I know some of them, because the Foundation had a role in their search for information and help.

Each year since 2005, the Bicuspid Aortic Foundation has chosen September as a special month - a time to raise awareness and bring attention to the aorta in the chest. We do so again this year. Why? Quite simply, thoracic aortic disease (TAD) is a killer. Despite efforts to estimate how many die from it, no one knows exactly how many there are. It should not be allowed to kill anyone.

Please help us, in September and every month, every day of the year, share information about thoracic aortic disease with others.

It could save their lives.

And if something is just "not right" in your chest, and doctors say it is not your heart, seek out someone with the expertise to check your aorta.

It could save your own life.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Phill Michael - Mountain Climber Born with Bicuspid Aortic Valve - Dies

2011-08-07T17:06:00.000-07:00

Infection presents particular risks for those born with bicuspid aortic valves. I remember being cautioned years go, when my husband's BAV was diagnosed and replaced, that at the sign of any fever we were to contact our doctor. Why? Because of the risk of infection to the heart - the medical term is endocarditis.

I remembered that today, as I read about a strong, vibrant man - Phill Michael - born with a bicuspid aortic valve. According to the press account, his BAV was replaced at the age of 32. He died July 30, 2011, at age 40, because of infection. http://www2.canada.com/topics/news/national/story.html?id=5217345

His friend, Chris Stanko, described what happened here: In Loving Memory: Phill Michael - 1970 - 2011 - an open wound, infection lodging in his heart, then reaching his brain. Doctors wrote about endocarditis long before there were any antibiotics to treat it. It is sobering that today, despite powerful medications, lives are still lost.

Phil's blog is a wonderful sharing of his life - scrolling down on the left, I found his Tears of Joy about his BAV replacement experience.

Those with BAV are often described as active and athletic - and it is very true. Looking at the pictures on Phill's blog, I am reminded of all the others with BAV I know - exceptional athletes in their chosen sports and activities. They are generally healthy and very high energy, but they have a defect - a "fish-mouth" valve in their heart - that makes them vulnerable to infection (before and after valve replacement), to heart valve failure, to aneurysms (aortic and brain).

Every time I learn of a loss such as this, I know there is much more to be done. At the Foundation, we invite you to join us in pressing for a better day for those with BAV, when the lives of these special people are no longer at risk.

My deepest sympathy to all who knew and loved Phill; it is clear as I read about him that he truly had a special heart.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Living with Bicuspid Aortic Valve Disease

2011-08-04T21:17:00.000-07:00

Carrie Mettler is a member of the Board of Directors of the Bicuspid Aortic Foundation. At the 2010 Conference, she spoke about what living with complex bicuspid aortic valve disease is like for her.

Echocardiograms - Using Sound Waves to Look at your Heart

2011-08-03T22:35:00.000-07:00

DeAnne Paul will tell you that she loves her job - and it is a very special one. She spends her days looking at hearts. DeAnne is like a very special photographer - called an echosonographer because instead of light, she uses sound waves to see inside the chest and capture the motion of the beating heart. Below we share DeAnne's presentation (Parts One and Two) from the Foundation's 2010 Conference.

Derek Owens - Hope for Teens with Bicuspid Aortic Valves

2011-08-03T21:33:00.000-07:00

Derek Owens loves basketball! Confronted with the need for surgery while still in high school, his dream was to play again - something that he had been told might not be possible.

In this video you will see Derek living his dream - back out on the court again. Thank you, Derek, for sharing your story and giving hope to us all.

Jen Lopez - Hope in Action

2011-08-02T22:24:00.000-07:00

It was in an ER in 2003 that Jen first realized that she had it too - the aortic condition that had already taken the lives of her father and grandmother. In the intervening years, Jen has faced life-threatening dissections and injury that ultimately required a heart transplant.

In the video below, Jen and Dr. Sharo Raissi, who opened many doors of hope for her, tell her story.

It is indeed a story of hope in action.

Contact Us

2011-08-01T21:51:00.001-07:00

Website: www.bicuspidfoundation.org

Phone: (949) 371-9223

Email: contactus@bicuspidfoundation.com

Mailing Address: 30100 Town Center Drive,

Suite O-299

Laguna Niguel, CA 92677

Donations

2011-08-01T21:50:00.000-07:00

The Bicuspid Aortic Foundation is a non-profit corporation with Federal tax exempt status under section 501 (c) (3).

For more information, please visit Donations and Volunteering

About Us

2011-08-01T21:46:00.000-07:00

Bicuspid Aortic Foundation Board of Directors

Bicuspid Aortic Foundation Mission Statement

The Bicuspid Aortic Foundation's mission is to:

• promote the well being and longevity of those affected by bicuspid aortic valve disease, thoracic aortic aneurysm and dissection, and any and all related conditions

• provide information and educational resources for patients, their families, and the general public

• encourage and support medical professionals and research scientists in exchanging information and advancing understanding of bicuspid aortic valve disease and thoracic aortic aneurysm and dissection, their underlying causes, and the optimization of care for these conditions

• seek research funding for bicuspid aortic valve and thoracic aortic aneurysm and dissection conditions

Join the Cause - Accurate Diagnostic Testing for BAV and TAD

2011-07-31T22:35:00.000-07:00

Recently I went with a family member to have an echocardiogram and the next day a CT Angiogram. These are among the tests currently available to look inside and "see" the heart, its valves, its arteries (the CTA), and the aorta. Since then I have had many thoughts about the "A" in the Bicuspid Aortic Foundation's ABC's - Accurate diagnostic testing - and the life-saving difference it has made in my own family and many others.

One important thing that individuals and families with Bicuspid Aortic Valve (BAV) and other forms of Thoracic Aortic Disease (TAD) can do is understand the tests that are available today to baseline and monitor their heart, its valves, and their aorta. At the Bicuspid Aortic Conference in 2010, the topic of imaging was presented. Following is a video clip from that Conference:

To the great physicians Abbott and Osler, who through diagnostic brilliance and keen observation via autopsy, understood and wrote a great deal about bicuspid aortic valves, aortic aneurysm, coarctation, and brain aneurysm, the diagnostic testing and surgical treatments of today were perhaps at most a dream, a glimmer on the horizon. Today, accurate interpretation of these tests are an important part of saving the lives of those with BAV and other forms of Thoracic Aortic Disease.

If you would like to support screening of young athletes for BAV, please visit

Together we seek to understand BAV and TAD,

As together, we create a climate of hope,

Arlys Velebir

President and Chair

Bicuspid Aortic Foundation