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

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"

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)

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. 

Aortic Dissection and Cardiac Tamponade

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

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

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"

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

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

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

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

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

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

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

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

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

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 - Familial Thoracic Aortic Disease

Jen and Her Dad - One Family's Story

Jen's High School Graduation
On this happy day, there was no inkling
of Thoracic Aortic Disease and what it would later mean.

Jen Lopez

Jen Lopez was at work, catching up on her email that morning, when she felt the first stirrings of something wrong. Mild at first, her symptoms progressively became worse.  It was hours later, in the ER, that Jen learned what it was. Her descending aorta had torn. The aorta is the largest artery in the body, coming directly out of the heart. The aorta is normally strong and elastic, but if its tissue becomes weak and fragile it is prone to tearing and rupture. There are families in which there are no signs or risk factors for a diseased aorta other than sudden deaths in multiple family members, often at young ages. This condition is called familial TAAD (Thoracic Aortic Aneurysm and Dissection). On the day it sent Jen to the ER, it had already claimed the lives of her Dad and her Grandmother.

Jen's Dad, Louie

In 1995, Jen's Dad, Louie, was 47 years old and in great shape. Outgoing, gregarious, and active, he often was found on the basketball court or the softball field. From all appearances, Louie could reasonably expect to enjoy many more years with his daughter, Jen, and wife, Terry. A successful oldest son, he was the center of his extended family, which included his mother, father, brother and sister and their families.

During a rare doctor's visit in September of that year to stitch up a soft-ball injury, Louie's doctor recommended a chest x-ray based on the fact that Louie had never had one as an adult.  The chest x-ray revealed a 5.6 cm aneurysm in his aorta and a previous Type B dissection in his descending aorta.         

Waiting for Surgery

Louie was referred to a cardiologist who recommended he wait to schedule surgery until the aneurysm had grown to 6.0 cm.  In the meantime, he was put on blood pressure lowering medication and instructed not to lift anything heavy. 

Six months later, while at his office on a Friday morning, he experienced searing pain in his mid-section and abdomen.  Within half an hour he was at the Emergency Room of The Queen's Hospital in Honolulu.  A CT scan showed a Type B re-dissection of the aorta (Louie knew nothing about the original dissection, including when it may have occurred).  This re-dissection went further this time, extending down into the abdomen, and the aneurysm had grown from 5.6 cm to 5.8 cm.  The cardiologist said he could not perform surgery yet because of the new aortic tear.  He wanted to wait at least a week, hoping for some healing during that time. 

Forty-eight hours later, in the hospital, as Louie was talking to the cardiac surgeon, the aneurysm ruptured.  He was rushed to the operating room, where three and a half hours of surgery could not save his life.  An acute Type A dissection of the ascending aorta had occurred concurrently with rupture of the aneurysm. The dissection disrupted blood flow to the brain and caused an extensive heart attack.

Five Months Later, Jen's Grandmother

Five months after her son's funeral, still reeling from this sudden loss, Louie's mother (Jen's grandmother), age 67, passed out on the grass while playing golf.  At the emergency room of a hospital she was pronounced dead.  An autopsy revealed that she too had died from TAAD. Like her son, Louie, her aorta had given way.

And then the family started remembering . . . .

             • Uncle Sonny, Louie's mother's brother. Dead at 53 from a sudden blood vessel bursting.

             • Papa, Louie's mother's father, father also to Uncle Sonny. 

               Dead at 55 reportedly of a burst blood vessel in his head.

             • And now, Louie and his mother.

Early, sudden death in a man, 2 of his 4 children, and his grandson - what was going on? There were no clear answers.  Some form of familial aortic disease was the reason behind these deadly events.

Very special Mother and Daughter
Their sparkling brown eyes give no hint
of all they have been through together.

Jen in 2003

Now we fast forward 7 years to 2003. Jen was living in the Washington, D.C. area, working for the Department of Justice, F.B.I., and traveling about half of each month.  In September she accepted a one-month assignment to Saudi Arabia.  Arriving back home on a Sunday in November, Jen was back in the office at her desk on Monday morning, but not feeling well. 

Thinking perhaps it was heartburn from the daily coffee, she ignored the discomfort, but soon experienced back pain, then, shortness of breath.  After several hours with no relief, her in-office medical personnel called an ambulance, and Jen was on her way to George Washington University Hospital (GWUH).   The pain never let up and the shortness of breath continued while ER personnel worked to understand what was going on. After several hours, a CT scan with contrast was ordered.  It revealed a Type B acute Aortic Dissection.  Jen's first thought on hearing that was, "That can't be right.  That's what my Dad had.  And he died." 

Jen's descending type B aortic dissection started just below the aortic arch and extended thru the abdominal aorta and on down into the left and right iliac arteries.  And, though she had never had high blood pressure before, there in the hospital her blood pressure and heart rate were high and hard to control, even with blood pressure lowering medication delivered by IV. Blood flow to Jen's kidneys was normal, although one kidney was being fed from the dissected portion of aorta.

Type B descending aortic dissections typically can be treated with medicine, not surgery. The initial concern was to control Jen's blood pressure and heart rate to prevent further propagation of the dissection and also prevent compromise of the renal and mesenteric arteries. In type B dissection, the hope is that with lowered blood pressure the dissection will not progress.

Four days after Jen's admission, an aortogram was performed. This procedure has historically been used to visualize the aorta, but is no longer used by aortic physicians, especially in cases such as Jen's. 

Jen stabilized and after 2 weeks in the hospital, she was released with instructions for care and appointments with a renal specialist. 

Chest and Back Pain - Again

Only 22 hours later, after just one night at home in her own bed, Jen was having lunch with her cousin and a friend when she started to feel ill. She collapsed on the sidewalk outside the restaurant with intense chest and back pain.  An ambulance returned her to GWUH, where cardiac catheterization revealed a new dissection, but not in Jen's aorta. 

This time, one of her coronary arteries, the left main, had dissected back to the 'mouth' of the artery. There was no blood flow in the anterior descending portion of the artery.  A surgeon patched the 'mouth' end of the artery with a pericardial patch and used an internal mammary artery to by-pass the anterior descending, restoring blood flow to Jen's heart.  

Heart Attack During Surgery

At some point during surgery, Jen had a myocardial infarction (MI), a heart attack. It was later discovered that it was centered in that lower anterior part of her heart where the blood flow was compromised.  

Jen survived the surgery but her recovery was complicated by congestive heart failure as a result of the MI.  The heart failure eventually necessitated the implantation of a Bi-V ICD (bi-ventricular cardiac synchronization device and defibrillator) in her left chest. 

But Jen is alive!  And over the next 3 years her health stabilizes.

Jen in September 2006

Three years later found Jen living with the dissection in her descending aorta, remaining on blood-pressure lowering medication and a heart medicine to battle the ongoing heart failure.   Because of her youth, the hope was that her heart muscle might yet re-model and grow stronger. Two of the four valves of her heart might need to be replaced eventually. 

Jen and Terry traveled to southern California in September 2006. It was intended to be just a brief visit. In addition to attending Bicuspid Aortic Foundation activities for TAAD Awareness 2006, Jen also had follow up tests and appointments. One of those tests, a 64 slice CT scan, revealed a new development - another dissection, this time in her other coronary artery. When told of the new tear, Jen and Terry knew when it must have happened. On the evening of August 31st Jen felt a slight pain in her chest. Locally, nothing was found. Without the fast camera and high resolution of the relatively new 64 slice CT, this new tear would not have been found. But now the approach to treating Jen had to change. She would not survive another tear in the arteries of her heart. Jen needed a new heart.

Waiting for A New Heart, December 2006

Jen was asked to remain in Los Angeles and undergo evaluation for a heart transplant rather than returning to Hawaii. A trip of a few days turned into months as she remained in southern California, waiting for a new heart. 

December 20, 2006

Jen ready to leave the hospital with her new heart

On the morning of December 20th, the phone rang. There was a heart for Jen. With that call Jen's plans for breakfast with Terry, whose plane from Hawaii had just landed, quickly changed. They would  meet at the hospital instead.

While waiting in a hospital room, someone asked Jen if she was nervous. She calmly said that this was so much easier than before, when she was rushed to the hospital in pain. For Jen, who has survived three dissections, this was the first time she would undergo surgery by choice, not as a life-threatening emergency.

Those who watched this brave young woman wheeled through the doors to surgery saw in that moment the same courage and gentle strength with which Jen has traveled the world, protecting the freedoms of us all.

Waiting on the other side of those doors was a new heart with healthy blood vessels and the skilled hands of the transplant team. Among the hands there to give Jen a new heart were those of the aortic surgeon who had steadfastly championed Jen's cause, determined that Jen and others with TAAD be given every opportunity to triumph over it.  

It seemed very fitting that it was in December, the month of Christmas and also her birthday, that Jen received the gift of life, a new heart. Somewhere, in the midst of their sorrow, may a family be comforted in knowing they have given the precious gift of life to another.  The heart of their loved one has a new home. It beats now in Jen's chest.  

  

 From great loss, there is also a new beginning . . . .  

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

Contact Us

Website: www.bicuspidfoundation.org

Phone:  (949) 371-9223

Email: contactus@bicuspidfoundation.com

Mailing Address:  30100 Town Center Drive, 

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                               Laguna Niguel, CA  92677