Sunday, December 16, 2012

Infection and the Surgeon's Hands

"Surgeon infected patients during heart procedure, Cedars-Sinai admits"

When I read this LA Times article, I thought of my husband's three open heart surgeries - two of them for the aortic valve, one for the aorta. I thought of the many others I know who have had aortic valve surgery and some who have had mitral valve procedures. And I thought of the many of all ages who look forward to heart valve surgery one day to extend their lives. When their heart valves fail them, surgery is not just an option, it is their only hope for continued life. What if the seeds of infection remain behind, inside their hearts, after surgery?

What Can Be Done to Avoid This?
What would it be like to go through this major surgery, begin the road to recovery, and then have the newly implanted valve and surrounding tissue attacked by a life-threatening infection? It is a road that no one would ever choose to travel. What can be done to avoid it?

Three things were mentioned in the LA Times article:

  • the "nature" of  valve replacements 
  • micro tears in the gloves
  • inflammation on the surgeon's hand.

There is a great deal of "sewing" around the valve that is necessary to anchor it securely, without leaks. That is the nature of the surgery. Given that, what can an informed patient do to increase their safety?

Micro Tears in Gloves 

Quoting the LA Times article linked above, " ...they made a surprising discovery: microscopic tears in the gloves typically worn by surgeons after performing valve replacement surgery. "

Others have investigated tears in gloves, which are important to protect the patient and also the surgeon. This paper from Germany about micro tears in gloves was published in 2010 in the the American Journal of Infection Control.

 Concentration of bacteria passing through puncture holes in surgical gloves.

Please note that these surgeries were laparotomies (abdominal surgeries), not heart surgeries. The authors noted that micro perforations went unnoticed by the surgery team 82% of the time

A previous blog touched on the importance of the surgeon's hands, raising awareness of the importance of the surgeon's skill 

 "In Our Hands" - Outcomes of Surgery.

Here, we raise awareness of the importance of something that began long ago with Lister - prevention of infection. 

The LA Times article further reports that "All surgeons doing valve replacements are now required to change gloves more frequently, officials said. Some surgeons are wearing double gloves during the operations, Sax said."

Asking Questions, Making Decisions
In raising awareness of what happened to these five people, others can learn from it and discuss important questions with their surgeon. Questions about the risk of infection.
  • What does the surgeon, and the hospital in which he operates, do to prevent infection?
  • If you are having heart valve surgery, do you know how often your surgeon changes gloves? 
  • Are double gloves used? Why or why not?
  • Would the surgeon operate with an "inflammation" on his hands? How are such decisions made?
In addition to asking questions, there are resources to help patients and families research their options for care, including information and increased focus on infection acquired in hospitals. As a start, the Leapfrog Group provides letter grade ratings of hospital safety through their Hospital Safety Score and there is also the Safe Care Campaign.

 There is no substitute for being an informed patient.
It is an important part of creating,
and living, in a climate of hope.

Best wishes,
Arlys Velebir
Bicuspid Aortic Foundation

Sunday, November 18, 2012

Where Are the BAV Children Now?

Sometimes the Bicuspid Aortic Foundation receives email like the following. It makes us feel very sad and concerned. Please note that the letter below is based on real experience. However,  some wording has been changed and all identifying information removed. Any resemblance to actual persons, living or deceased, is accidental only.

Dear Bicuspid Aortic Foundation,
My daughter was diagnosed with a bicuspid aortic valve when she was 15 years old. I still have the report of her first echocardiogram, which says she had mild to moderate aortic insufficiency. She had a second echo three years later. That was the last echo she had.

My problem is that my beautiful daughter has always been quite active and has never felt any obvious problems from her heart. She never took this issue seriously, I guess, until lately. She feels fatigued, her legs and ankles swell so badly, sometimes she is unable to pull her jeans over her legs. She has pressure in her chest. Generally, she just feels very badly. I am so concerned. She is now 36 years old, and her children need her. 

A Worried Mother

In 2007, physicians from London's heart hospital wrote about two men with BAV, raising a similar concern in this paper: An undiagnosed bicuspid aortic valve can result in severe left ventricular failure .

The first man was described as follows: "A 48 year old builder presented with shortness of breath at rest and mild icterus. He had been prescribed antibiotics for a presumed chest infection when he visited his general practitioner six months earlier. A heart murmur was noted during that consultation but not followed up. He later recalled that as a child he underwent annual review for a heart murmur. At age 16 he was reassured and discharged from clinic." What happened to this man? He could not wait for a scheduled surgery date, because his liver and kidneys began to fail. He survived the surgery (the BAV taken out was so calcified, the opening was described as a "pin hole"), but his left ventricle had not recovered its function when the paper was written. Permanent heart damage, at the age of 48. A tragedy.

The second man's story begins "A 46 year old postman was admitted with increasing shortness of breath on minimal exertion (New York Heart Association functional class III). In early childhood, he had been diagnosed with a heart murmur and followed up in a tertiary centre. At age 10 he was either discharged or stopped attending appointments." What happened to him? "He underwent urgent aortic valve replacement during that admission. The ascending aorta measured 5 cm intraoperatively and was therefore also replaced."

From the authors' discussion, "Our cases highlight the potential complications of a bicuspid valve, which could have been prevented by regular monitoring and earlier surgery. Avoiding urgent and emergency surgery has advantages in terms of surgical mortality (perioperative mortality for first time aortic valve replacement in the United Kingdom: emergency surgery 12%; urgent 5%; elective 1.9%; personal communication, Bruce Keogh, Society of Cardiothoracic Surgeons and AD Cunningham, Central Cardiac Audit Database) and the longer term effects of ventricular impairment. Bicuspid valves are present in many people who were reassured in early life or lost to follow-up and in many “healthy” middle aged men whose hearts have never been examined."

This baby grew up to have an experience
similar to the builder described above, with
one  important difference - his heart fully
 recovered after valve replacement surgery.
The message is an important one for adults - if you remember being told about a heart murmur or bicuspid aortic valve as a child, don't wait for symptoms. Get checked as soon as possible, before your heart is permanently damaged or your aorta causes an emergency.

And for parents of children with BAV or even seemingly innocent heart murmurs, do all you can to be sure they are not "lost to follow up". It just might make all the difference between disability, even death, and continuing a normal, active life.

Best wishes from the Bicuspid Aortic Foundation

Saturday, November 17, 2012

Heart Angels

The greatest achievement was at first and for a time a dream.

The oak sleeps in the acorn, the bird waits in the egg,
 and in the highest vision of the soul a waking angel stirs. 

Dreams are the seedlings of realities.
                                  -James Allen

Dreams. What Are Our Dreams?
We dream of a time when every newborn has an aortic valve with three perfect leaflets, free of risk from infection.

We dream of a time when the aorta in our chests no longer weakens, thins, bulges, tears, ruptures . . . and our head and neck vessels are perfectly healthy.

Until that day, we have some other dreams.

We dream of no more sudden death, no more injury.

We dream of perfect BAV repairs and perfect replacement valves.

We dream that the "pictures" taken show clearly what is happening inside our chest.

We dream that we are believed when we say we are not anxious, that something is really wrong!

We dream that physicians know who needs their aorta replaced, and exactly when it should be done.

We dream of programs and centers that specialize in the lifelong care of BAV families.

We dream of support, compassion, and understanding for those with BAV/TAD everywhere, through out their journey through life.

Heart Angels Turn Dreams into Reality
After centuries of causing suffering and death, the bicuspid aortic valve is beginning to receive much needed attention. Our dreams will become reality with the help of angels, stirred in their hearts, to make it happen. Donations can be made through the Bicuspid Aortic Foundation Razoo Page 

or via check to:
                                  Bicuspid Aortic Foundation
30100 Town Center Drive, Suite O-299
 Laguna Niguel, CA  92677  

Monday, November 5, 2012

Bicuspid Aortic Valves - Our Time Has Come!

Dr. Sharo Raissi, Los Angeles,  Dr. Arturo Evangelista, Barcelona,  Arlys Velebir, Laguna Niguel,  Dr. Simon Body, Boston
An Extraordinary Lunch 
It must have looked so ordinary - four people around a table in Los Angeles, having lunch together.To the casual observer, there was nothing to suggest anything special or unusual. The server may well have wondered at how little attention was given to menus, how intensely they spoke together, and how long they lingered there.  Tucked away in the very back of the restaurant, this was no ordinary group. This was no ordinary lunch.

This Is Our Time!
From different backgrounds, different parts of the world, they had carefully planned to meet here. Just what were they talking about, perhaps even plotting? Something extremely important had brought them together -  what was it? Nothing less than an all out assault on a source of great human suffering - the bicuspid aortic valve. After centuries of pain and suffering, the time is right. The time of the bicuspid aortic valve has come!

Who Were These Four People?
Dr. Simon Body, an anesthesiologist, practices at Brigham and Women's in Boston. Dr. Arturo Evangelista practices cardiology in Barcelona, Spain. Dr. Sharo Raissi, a cardiothoracic surgeon in Los Angeles, chairs the Scientific Advisory Board of the Bicuspid Aortic Foundation. I was deeply touched by their humility, compassion, and courage in the face of a complex condition for which there are no easy answers. 

I was the fourth person there, at that table, in your place - you, the millions of families everywhere with BAV. As I sat there, I thought about so many - my beloved husband, our family, and the great world-wide family with whom we are linked through this common genetic heart defect. I told them some of your stories - the tragedy and the triumph. 

The First International Consortium on BAV
At the Foundation, we speak of the world-wide tsunami - the millions with BAV and their untold number of relatives with aneurysms. Yes, it may be a tsunami, but there is a tremendous force gathering to address it. This marks the first time that centers are collaborating internationally, organizing together to address BAV. Participation in this great work is not a light thing - it calls for commitment over many years and unwavering determination to find answers. There is no misconception that it will be easy, and yet there is great enthusiasm among those signing on to this effort.

Finding the Answers
Think of all the unanswered questions there are about bicuspid aortic valves and the families in which they occur. Yes, we live in a day when testing, surgery, and blood pressure medications are saving lives. But there is still a tremendous, unmet need in BAV families.

Most of the mystery, and far too much misery, remain.

We Cannot Change the Past - Let's Change the Future!
For many of us, if there were a time machine, we would return to a time before our loved one was injured, before we lost them. We would not ask for extra time, just enough to get them the help they needed. For others, even if we could go back in time, there would be no help. Some things are still beyond our reach today.

We cannot go back.

But we can do something. 

We can change the future.

This is an exciting time. We need your help to change the future. In upcoming posts, we will let you know how you can help.

Best wishes to you and your family,
Arlys Velebir 
President and Chairman
Bicuspid Aortic Foundation

Saturday, October 6, 2012

Bicuspid Aortic Valves - the View from Those Who Have It!

Survival and BAV
From time to time, bicuspid aortic valve studies are published and articles appear in the press, such as this one from 2008, Adults With Aortic Valve Disorder Do Not Experience Reduction In Survival Rate, which mentions following 642 adults, average age 35, for 9 years. 

It is encouraging to read that advancements in surgery and medical technology have helped those with BAV, as mentioned in the article. However, it is also important to understand the BAV experience through those who live it, as long as they live. Robby Benson was born with a bicuspid aortic valve. Four heart surgeries later, he is telling the world about his life with BAV, including this October 4, 2012 article in People Magazine, Robbie Benson: Inside His 'Brutal Experience' of Four Heart Surgeries.  

Beyond Survival
It is important to remember that beyond survival lies the goal of living life to the fullest - something that those with BAV may be denied at some point in their lives. There is a great deal that is not understood about those with bicuspid aortic valves and their families. With time, some of them develop additional complications.

It is nearly 7 years since my husband was injured when fine, hair-like strands of tissue broke away from his mechanical valve and injured the right side of his brain. A survivor, yes, even a miracle, but the life he once knew cannot be fully restored. 

Not long ago, along with a bulging aortic aneurysm, a mechanical valve was removed from a man with BAV - it had strands on it too. I was so glad it was done before his brain was hurt. But it was the second surgery for that man in just three years. Survivor? Yes, but can't life be better for those with BAV?

Remember - There are Those Who Don't Make It
This year I spoke with a man in his 70's who is a true survivor - aortic dissection first and a few short years later replacement of his BAV. Yes, another miracle man! He told me about going to the hospital to visit a young man with BAV who needed a second surgery. This was a young man who had supported him during his own dark times. Calling from the hallway outside ICU, he dropped the phone in shock - his friend was dead. 

And lest we think children are not affected, here is Rylan's story. From the local newspaper, Orion boy, 4, loses quest for new heart, and a memory video here, Rylan Foster. Rylan was born with a bicuspid aortic valve.

Through these stories, may we remember the millions born with BAV.
Among them are those who are delicate and vulnerable, 
who need help that even today may be beyond reach.

May we be their voice, 
creating a climate of hope.
Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Sunday, September 30, 2012

Genetics and Bicuspid Aortic Valve Families

Bicuspid aortic valves have been known for centuries, appearing in the anatomy studies of Leonardo di Vinci. 

The advent of antibiotics, open heart surgery, and aortic surgery brought hope to those born with bicuspid aortic valves and their families.

However, there are still many questions that have no answers. Recently it was announced that a group of centers around the world are joining together to study the genetics of the bicuspid aortic valve. You can read more about it here, the BAV Genetics Newsletter.

Despite how much progress has been made, there is still pain, suffering, and death experienced in BAV families. The Bicuspid Aortic Foundation looks forward to the day when there are more answers, more understanding, and a better life for the millions who live with BAV and their tri-leaflet aortic valve family members who may be affected by aortic aneurysm and dissection.

We cannot change the past,
but we can change the future!

The collaboration surrounding
 the BAV Genetics work is an important beginning,
 creating a climate of hope.

Sunday, September 23, 2012

Victory Over Aortic Aneurysm - the OTHER KILLER in the Chest

Something Is Terribly Wrong!
Sandra on Barker Mountain, 2012
Sandra Croff knew something was wrong, but no one had been able to find it. She had tremendous fatigue, shortness of breath, chest pain, and at times felt her heart doing flip flops. 

After seemingly endless doctor visits and tests, she was told that her heart was fine. She was just suffering from post menopausal symptoms and anxiety.

Among those many tests, Sandra had a simple chest x-ray. She remembers looking at it with the doctor, and asking him "What is that?" When told it was her aorta, she asked the doctor if it should be so large. The doctor dismissed it, saying her aorta was fine, and proceeded to tell her she must have fibromyalgia. Sandra firmly told the doctor, "I do not." She turned and left that office, disappointed again. 

Sandra remembers one particular day very well. She had horrible pain on the left side of her chest, going through to her back, and into her arm. It lasted for about three hours. Sandra did not go to the ER that day, because based on past experience she felt no one there would believe or help her. But she knew she had to keep looking for answers, because others in her family had died suddenly, supposedly of massive heart attacks.  She wondered if her own life would be cut short before she found anyone to help her. And she also wondered, even if she lived, if she would ever feel well enough to do even simple things she loved again without labored breathing and a stabbing, burning feeling in her chest. 

Sandra, 7 days after aortic surgery
In 2010, Sandra made a call to a long-time family friend. She had heard that he had some kind of heart surgery, and she wanted to know the details. That family friend was Bob Gies, and before long Sandra traveled to the same aortic treatment program. There she had the testing she needed. It showed that Sandra had an aortic aneurysm in her chest. She began to make plans for surgery.

Something is Now Very Right!
Sandra now has her life back! Always active, Sandra proved that her problems were not related to declining hormones, anxiety, or fibromyalgia, but due to faulty "plumbing" in her chest. Below, in her own words, Sandra describes hiking Barker Mountain recently.

Hiking Barker Mountain, 2011
Hiking Barker Mountain, September 2012, in Sandra's Own Words
I was in Montana this week and hiked to the top of Barker Mountain again. The day started out at a cool 42 degrees, and some of the rooftops had frost on them.  My hike began at the bottom of the mountain, just where the road splits and Green Creek crosses.  Arriving at the starting point, I had to get my boots on, so that I could embark on this steep outing. It was about 11:30 am as I started to walk and also take pictures of the new survey, being done by the Forest Service. 

As I crossed Galena Creek a couple of times, I could feel the steep grade tasking my body, not only my lungs but my legs.  I felt my heart beating faster and louder.  I am from a part of the country that is only about 150' above sea level.  These people in Montana are like mountain goats, as far as their lungs and their capability to walk this steep terrain!  I guess I am a flat lander? When I reached one stretch that is much steeper than the rest, I would stop briefly and have a drink of water.  

Much further up the mountain, there is a long stretch where the cool breeze comes down the narrow roadway.  It refreshed my mind, and also rejuvenated my body.  The breeze along with the sweet smell of the pines is just beyond description.  It is the type of breeze that makes you smile with pleasure.  Oh, how I love the breeze!  As a child I lived in Southern California, and in the morning it would be cool to chilly, and the breeze would come from the Pacific Ocean to energize you. The last point where you feel this breeze on the mountain is situated just below where the sun hits the side hill.  From that point on you are in the sun completely.  Knowing this is the last pull to get to the top, I am telling myself, "You are almost there!" and "Get moving!". 

After getting to the top, a large rock was the perfect place for lunch.  I was out of steam at that point! After eating and drinking water, it was time to start the descent. Of course this is the easiest part of the hike, however the ground has unstable rock on it and care is needed to avoid sliding.

It was indeed a body and mind builder.  On the way down, a drink of fresh, cool water from Daisy Creek was indescribable.  This small creek comes from higher up the mountain and is the best water around.  It is very cold, and thirst-quenching. 

This was the second time I have hiked this mountain since I had my aortic surgery. 
Of course, being 64 years does not say you will be able to do everything you desire but your mind allows you to achieve what you want, especially if your heart is fit. It did my heart good to make this hike again this year. As I was driving down the road, I was able to see the mountain in my rear view mirror, and all I could think of was next year's hike!

Saturday, September 8, 2012

Robby Benson Shares His Life with Bicuspid Aortic Valve Disease

From the first words of the Preface, I felt that I knew Robby Benson, although we have never met.

Is it because he is almost blind
 without his contact lenses?

Is it because a childhood
heart murmur  was not
"innocent" after all?

Is it because a loving young 
couple with a small child
faced life-and-death surgery
 much too soon?

Is it because it hurt so much
 when those chest tubes were pulled?

Is it because no one listened, 
no one believed him
after his second surgery, 
when he could not breathe?

Is it because his chest 
has been opened four times?

Is it because they did not hold his hand, 
did not comfort him, until finally,
there was someone with compassion
during that fourth surgery?

Is it because of the moving description of
a life-long quest for help,
searching for a place of hope,
by someone born 
with a bicuspid aortic valve?

Yes, it is because of all those things and more. 
You see, my husband was born with a bicuspid aortic valve, too. He has had three surgeries. And over the years, through the Bicuspid Aortic Foundation I have met many others. And yes, they are all very special. They are active, vibrant, gifted men, women, and children, with something special in their hearts. Their aortic valve has only two leaflets, and often their aorta is larger than normal, potentially bulging until it becomes an aneurysm. Their family members, with all three aortic leaflets, may be at risk of aneurysms too. Not everyone has the same characteristics, but some things, including the near-blindness, are not uncommon. On the Foundation website page, Bicuspid Aortic Valve, we describe these characteristics under "The Big Picture"

They can live extraordinary lives,
 making wonderful contributions to our world.
 But they also need help, they need hope.

Here are links to Robby's book:

Thank you, Robby, for opening your heart and sharing your life,
Creating a climate of hope,

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Monday, September 3, 2012

"In Our Hands" - Outcomes of Surgery

"In Our Hands"

When surgeons speak about their results, they often use the phrase "in our hands", graciously crediting the entire surgical team. 

Their patients think a great deal about hands also, more specifically about one pair of hands, the hands of the surgeon. 

We wonder if surgeons realize how much we look at their hands when we meet them. Our minds are racing - are these the right hands to open my chest, touch my heart, replace my valve, remove my bulging aorta? Can I trust these hands?

Practice Makes Perfect
Common sayings are rooted in practical life experience, and the phrase "practice makes perfect" is no exception. The more we do something, the better we get!

Outcomes of Aortic Valve Replacement Surgery
In 2003, the New England Journal of Medicine published a special article, Surgeon Volume and Operative Mortality in the United States.  It was a report on the outcomes of 8 surgical procedures in Medicare patients. One of  those procedures, aortic valve replacement, is important to those with aortic disease. There was further discussion about it in this article also, High surgical volume equals better patient outcomes. Looking at a chart in the NE Journal article, (Table 2), the affect on outcome of the hospital's volume for aortic valve replacement was 0%. The affect of the surgeon's volume on outcome was 100%. 

In other words, it was all about the surgeon's hands.  

Asking Questions
That is why it is so important to ask certain questions of the surgeon. Make a list! How many bicuspid (or trileaflet) aortic valves have you replaced (or repaired) this year? Last year? Last five years?  How many aortic aneurym procedures? How many of this exact procedure did you do this year ( last year, last five years, etc.)? (Bypass results are not of interest unless bypass surgery is needed, so be careful to avoid general totals of all heart surgery.) How many people died in the hospital? How many died within 30 days? How many were injured or had complications? What were they? How many had infection? Bleeding? How many needed permanent pacemakers after surgery? How many needed heart medications long term that they did not need before surgery? The Foundation Support Page has additional information that may be helpful.

Listening Carefully to the Answers
The Foundation has heard from some patients who believe they did not receive clear, straight forward answers. Clear communication can be difficult in many settings, but especially so when talking about your own major surgery. Take someone with you. 

For example, if you ask how long a certain valve has lasted in others, listen carefully for general answers such as "I have never had to take one out." Follow up with more questions. Find out if the surgeon is talking about a clinical trial that has been going on for a few months, or a valve that has been used for many years with an established track record. That background can put a whole new meaning around the word "never".

Do your best to avoid things like these that have happened to others:
  • A woman did not realize she was part of a clinical trial until the hospital contacted her months later for a "free" echo of her prosthetic aortic valve.
  • A man arrived home after surgery thinking he had received a certain kind of tissue valve. He had specifically discussed and requested that kind of tissue valve when talking with the surgeon. He was more than a little surprised to discover later that in his chest was a different kind of tissue valve, from a different manufacturer. No one had told him while he was in the hospital. 
  • A man, still in the hospital, was shocked when the technician told him during his echo that he had a beautiful mechanical valve. He and his family were sure he had requested tissue. No one had told him anything about receiving a mechanical valve until the technician made that comment. If there were technical reasons for the mechanical, no one had explained it to him or his family.
  • A woman thought that her ascending aortic aneurysm was replaced with Dacron. When she began to have problems a few months later, in reading her surgery report she discovered that Dacron was not used at all. Her aorta had simply been "shortened". It put her in life-threatening jeopardy as the stitches holding her stretched aorta pulled away from  a newly implanted valve.
  • A man who had previously had his bicuspid aortic valve replaced required another surgery for a large ascending aortic aneurysm. When opening his chest, his aorta was punctured, causing massive, uncontrolled bleeding. He survived the crisis, but after surgery contracted numerous infections. After fighting for many weeks in the hospital, he lost his battle.
Would it have been different for them in other hands? It is an important question to ponder. 

Surgeon's Statistics
Statistics may be available if you search for them. Some states provide a public database of surgical results, however those results may only be for the most common heart surgery performed, bypass surgery. Search and see what you find.

Grateful for Pioneering Hands
Dr. Randall Griepp and Dr. Sharo Raissi

Dr. Randall Griepp, Dr. Teruhisa Kazui, Dr. Sharo Raissi
The phrase "in our hands" takes on special meaning as we think of the great pioneers in aortic surgery.  Dr. Randall Griepp for many years has advanced treatment of the aorta in the chest. His hands have pioneered many lifesaving procedures, taking on the most difficult challenges in the chest from the early days of heart transplants to circulatory arrest and the aorta.

Under his guidance, Mount Sinai developed a rich data resource of results over the years, forming the basis of many important, highly regarded papers on thoracic aortic disease.

The Foundation is grateful for Dr. Griepp's work, and that of other aortic surgeons we have honored, Dr. Nicholas Kouchoukos, and Dr. Teruhisa Kazui. Their work has saved many lives around the world.

Arlys Velebir and Dr. Nicholas Kouchoukos
When it comes time for surgery, may we all do our best to find expert, skilled hands in which to entrust our lives.

Choosing your Doctors and Hospitals


Give a man a fish, and he will eat for a day.

We Have Something in Common
In some ways we are alike, in others we are different. But we all have something special in common in our chests - aortic valve disease, aortic aneurysm, aortic dissection. Sometimes, we have brain aneurysms too.

And so, we have something else in common.
 Decisions about who to entrust with our medical care.

Critical decisions.
Too critical to rely on a simple list of names. 

My Family Needs Help! Who Is the Best?
Like most people, we turned to local physicians when my husband became ill the first time. We learned that he was in heart failure due to a severely narrowed bicuspid aortic valve. At that time, we had no way of researching our options.

We simply went from the internist to the cardiologist, the cardiologist to the surgeon. You may find it hard to believe, but we did not even meet the man who would open his chest and touch my husband's heart until the night before surgery, already admitted to the hospital.

Years later, the internet had emerged when the aneurysm was discovered in his chest. This time I knew there was no one in the local community equipped to understand and help. But there was still very limited information. There were just scattered crumbs along the way that we used to reach the hands of a skilled aortic expert.

As I write this in 2012, we still do not have all the tools we need to help families like my own research aortic disease in the chest. But we do have some - and we can use them to find help.

Researching Your Options Learning to Fish!
The Foundation is often asked about finding physicians with expertise in aortic disease in the chest. Until recently there have not been many options - just a handful of major centers in the US pioneered aortic surgery over the years, while heart surgeries in most communities were bypass surgery, along with some heart valves.

When it came to bicuspid aortic valves, it was considered simple, something that did not need specialized attention. That is just beginning to change.

This is where the fishing lessons come in - we all need to learn how to find the medical expertise that can help ourselves and our families. It will continue to evolve, new expertise and capabilities will emerge. How will we know who to trust with our lives? It is not as simple as a list of names. We need to do our homework. The Bicuspid Aortic Foundation has updated it's  Support Community Page - there are some ideas there to help you!

                             Teach a man to fish, and he will eat for a lifetime.                    

Bob Gies Learned to Fish and You Can Too!
Look carefully and you will see the fish on Bob's line in those clear Montana waters! When he began to have symptoms, Bob visited several cardiologist and surgeon offices, looking for help, not satisfied with the answers.

Today, he has both local care and follow up as needed with a specialized bicuspid aortic valve program. But he had to search to find it!

Bob has care not just for one day, but for a lifetime.
He knows how to be an active partner in his own health.

When Bob approached the Bicuspid Aortic Foundation, he did not just receive a fish for one day. He learned to fish for a life time. Today, he volunteers with the Foundation, reaching out to others. He knows they too can learn to fish!

This is an important topic for us all. Please check back for additional future stories to help you in your search.

Best wishes from my family to you and yours,
Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Saturday, August 25, 2012

Aortic Dissection in the Cath Lab

Dissection in the cath lab? 
Picture courtesy of
Are they studying frogs in there?

Most people associate the word "dissect" with the lifeless little frog they cut apart and studied in biology class. An online search of the word "dissect" describes a separation or cutting apart. Whatever does this have to do with hospitals and cath labs?

If we think about our blood vessels at all, we probably think of them as simple tubes of tissue through which our blood flows. Nothing about our bodies is that simple, and these vital highways through which our blood travels are no exception. 

The aorta is the largest artery in our body. One of the wonderful things about it are the three layers that make up the wall. No doubt there are many reasons for those layers and what they do. But sometimes something goes wrong, and the inside layer tears. If that were the only layer, blood would pour out into the body, and life would end in moments. But the aorta has two more layers, the outside layer being the strongest. With that outer layer holding, there is hope. If we picture blood inside the wall, pushed under pressure as the heart beats, the blood is separating the layers of the aorta. And this is where the word dissect comes in; there is a separation of the layers of the aorta - dissection of the aorta, as described by this medscape link.

So that is it - nothing to do with frogs at all! It is a gruesome thought that our own blood, powered by our heart, is tearing apart our largest blood vessel, separating the layers of our aortic wall.

Torn Aorta Detours Through the Cath Lab
They do study something in the cath lab - typically the arteries of the heart. They are looking at these arteries to see if they have any blockage - commonly called heart disease. "Cath" stands for catheter, which is threaded into the arteries of the heart, where x-rays pictures of arteries high-lighted by dye are taken. This is the angiogram or heart cath.

But sometimes, while looking at the arteries of the heart in someone with chest pain, they may discover the heart is fine, but there is something else - a torn, bulging aorta. It is one of the most critical, dangerous discoveries in the human body - terrifying for medical professionals, patients, and families. Getting the person out of the cath lab and into the operating room is the only hope for someone with Type A dissection. Sometimes, they make it against all odds. Sometimes, they do not.

At the Bicuspid Aortic Foundation,
 we call aortic disease "the OTHER KILLER in the chest". 

Heart disease is the number one killer of both men and women. But the diseased aorta kills too. Because of the confusion with heart disease, the number of people it kills is not known. What about those deaths thought to be heart attacks, but unconfirmed by autopsy or other clear evidence?

When the aorta is torn and bleeding (dissection), there is no time to be lost. Greater awareness of the OTHER KILLER in the chest can help medical professionals, patients, and families think about and find it, ideally without taking detours to the cath lab, looking for heart disease.

Heart disease is not the only killer.

 Remember the diseased aorta,
 the OTHER KILLER in the chest.

Saturday, July 21, 2012

Endocarditis and Heart Attack

A Rough Start to 2012
A new year begins with many good wishes for happiness and good health. However, as January 2012 began to unfold, it brought a crisis to my friend, John.

It was in January just five years earlier that John's bicuspid aortic valve and ascending aortic aneurysm had both been replaced. All had been well since, and he had no particular reason to imagine that just a few days into this new year would find him not only hospitalized twice but undergoing emergency surgery to save his life.

One evening John began to run a fever. Remembering the warnings he had been given about the dangers of infection for someone with a prosthetic aortic valve, he went to a hospital ER the next day. By the time he was checked there, he was no longer feverish and was sent home without further investigation. Two nights later, he was back in the same ER, this time with a soaring temperature of 105. He was admitted, a transesophageal echocardiogram (TEE) confirmed endocarditis, and he remained in the hospital on IV antibiotics for about a week.

What is Endocarditis?
In this video, after discussing infections more generally, beginning at 4:08 there is a TEE image followed by the actual surgical tissue from someone who developed infection on his bicuspid aortic valve.

Released Home - Crushing Chest Pain!
John was allowed to return home from the hospital with a pic line, through which he would continue to receive IV antibiotics. What a wonderful feeling to come home! But suddenly, just two hours after he got there, John felt horrendous chest pain and nausea. Something was terribly wrong. The call to 911 brought EMTs quickly. John had all the signs of a major heart attack, and his only chance of survival was to get to the nearest cardiac hospital. Now!

What Was Going On? Heart Attack?
Heart muscle needs blood in order to remain healthy. It will die without it. This is the searing pain of a heart attack. But how could John be having a heart attack? It was clear his heart was not getting the blood supply it needed, and nitroglycerin was not helping open up the blood flow. What was blocking the blood flow to his heart muscle?

At a nearby hospital, the villain was exposed - some of the infected tissue, called vegetation, had broken off and blocked his coronary artery, preventing blood from flowing to his heart muscle. John was indeed having a heart attack and needed surgery immediately.

It was already late in the day as the cardiac surgical team assembled. It would be a long night. After many hours of surgery that included carefully cleaning out the infectious debris and giving him a new prosthetic aortic valve, John was alive! Fragile, with injured heart muscle, but alive! 

There are some cases that heart surgeons particularly talk about and remember. These are the times when without warning they are called to wage tremendous fights, to save life against the odds. John became one of those cases. He eventually was transferred to the original treating hospital, where he continued his recovery.

Teeth Cleaning, a Cut on the Hand?
Looking back, John has tried to identify where the infection might have begun. There are two things that happened prior to his fever that are suspicious to him. He had a deep cleaning of his teeth performed, and although he did pre-medicate with antibiotics, was that sufficient? Around the same time, he also received a cut on his hand when he fell from his bike.

Always on Guard
Over twenty years ago now, when my husband received his first prosthetic aortic valve, we were warned to take any fever seriously. Although we knew he had a heart murmur, we did not realize he was at risk of infection before then. Over the years with his prosthetic valves, any hint of fever has always been taken seriously by his physicians. Invasive procedures have been accompanied by intravenous antibiotics.

What happened to John is a reminder of the special dangers of infection for those born with BAV.  In addition to damage to heart valves, endocarditis is associated with stroke. A shower of vegetation particles can be sent to the brain, damaging multiple areas. And as John's experience shows us, heart attack can also result.

Knowing About Yourself and Your Loved Ones
John was well informed about BAV and TAD, and he had shared that information with those close to him. In critical condition, arriving by ambulance in a hospital where no one knew him, precious time was saved because they could tell the EMTs and ER physicians John's story. 

John survived against tremendous odds. As someone who knows John said to me, "I'm just glad he's still here."

Thank you, John, for letting me share your story,
telling others of the danger of infection,
and helping create a climate of hope.

Arlys Velebir
Bicuspid Aortic Foundation

Sunday, July 15, 2012

Aortic Aneurysms, Bicuspid Aortic Valves, and The Sword of Damocles

Sword of Damocles, Richard Westall, Ackland Art Museum
"...the presence of a bicuspid aortic valve appears to indicate, at least in a portion of the cases in which it occurs, a tendency to spontaneous rupture of the aorta, which hangs always, like a Sword of Damocles, above the unsuspecting subjects of this type of coarctation, for this anomaly occurred in quite half the cases so terminating." M.E. Abbott, Coarctation of the Aorta, The American Heart Journal, 1928.

Among those born with bicuspid aortic valves are a group of individuals that are athletically gifted, with tremendous focus and stamina. They pursue amazing athletic goals with exceptional physical strength, endurance, and intelligence. They appear the picture of health, and in many ways their bodies are in excellent condition. However, life-threatening danger may hang over them, sometimes undetected. I recognized those traits in John Marschall, known as Coach Jay, who is interviewed below.

I was struck again by Dr. Abbott's wisdom, published over eighty years ago, as I read about this man's experience:  Go Hard or Go Home: Determination Despite Cardiovascular Disease .  However much others might admire his extraordinary athletic ability, no one would wish to walk in his shoes between 2008 and 2010, years when he first had his bicuspid aortic valve replaced, later contracted endocarditis, and then went under the knife again when a large aortic aneurysm was discovered. Coach Jay tells about his last race, and new beginning, in this post, Monday, June 27, 2011, The end and the Beginning . 

In Dr. Abbott's day, surgery and antibiotics did not exist. The sword, hanging by only the thinnest of threads, was out of reach and would eventually fall. Today surgery and antibiotics do exist, but the sword still hangs there, the dangers remain for those with BAV and TAD. There is still a great deal that is not understood. Controversies exist about the timing and methods of treatment and surgery. Individuals, families, and physicians have a great deal to ponder as they make decisions about first finding the sword, and then deciding when and how to remove it. 

Seemingly healthy, 
extraordinarily gifted people
 with BAV and TAD
 may be under life-threatening risks.

As we share their stories, 
we expose those risks 
and raise awareness,
creating a climate of hope.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Sunday, May 27, 2012

New Members of the Scientific Advisory Board

The Bicuspid Aortic Foundation is pleased to announce two new members of our Scientific Advisory Board:
Jason Sperling, MD, FACS, Subspeciality Director of the Thoracic Aneurysm and Bicuspid Aortic Valve Program at Valley Heart in Ridgewood, New Jersey, and Cheryl Maslen, PhD,  Professor of Cardiovascular Medicine and Molecular and Medical Genetics in the Oregon Health Sciences University School of Medicine, and Associate Director of the Heart Research Center there.

We would like to thank each of them for their work in these areas so vital to those with BAV and other forms of thoracic aortic disease. We are deeply grateful for their desire to voluntarily assist the Foundation in fulfilling its charitable mission.

Saturday, May 19, 2012

Find It and Fix It First!

In the ER
Crushing chest pain sent him to the nearest emergency room. His blood tests and EKG were normal - no sign of a heart attack. Maybe it was a bad case of GERD? They gave him a common antacid and admitted him for observation.

In the Hospital Room
The next day, arrangements were made to send him by ambulance to a hospital run by his HMO. They were just waiting for the ambulance to come.

If you were in the hospital that day, you might have wondered who was in trouble as Code Blue echoed through the hallways and waiting rooms.

It was this man, thought to have a problem with his digestion. His heart had stopped.

The nurse that got there and began CPR was strong, the chest compression forceful. His heart began to beat again.

In the Cath Lab
How had they missed this last night? Surely, he must have a blockage somewhere in the arteries in his heart. He was rushed to the "cath lab", where a catheter was threaded from an artery in his groin up into his heart. Squirting dye into his coronaries showed beautiful, clear, healthy blood vessels. No blockage there.

Then the cardiologist squirted dye into the aorta. And there it was, a tear in the ascending aorta.

And then, his heart stopped again.

If you were in the hospital then, you might have heard this over the loudspeaker: "Code Blue, Cath Lab!  Code Blue, Cath Lab!" And you might have wondered whose life was in danger.

They worked desperately on him, trying to get his heart to beat. At the same time, emergency calls went out for heart surgeons.

Somehow, his heart began to beat again.

When Every Millisecond Counts
It was the end of the day. One heart surgeon was in his office. Another, already headed home, was stuck in traffic.

There was a rush to assemble a team for the operating room. A thoracic surgeon will pitch in to help until the second heart surgeon can get there. They have to move this man from the cath lab to the operating room - will his heart keep beating?

His heart has already stopped twice. Everyone knows now that he is bleeding inside. His heart must keep beating. But every time it beats, he may be tearing and bleeding more.

Where is all that blood going? How long can he hold on?

Yes, they know he is bleeding and his aorta is torn. But rather than clear pictures from a CT scan for surgeons to look at, they have only the angiogram images. There is no time for more tests now. Not just every second, but every millisecond counts.

There is very little hope in this picture. Someone must explain to the family that the survival statistics are grim.

In the OR
On the table in the OR now, his heart still beating, the surgical team began their work.

For the third time, his heart stops.

They had given heparin, but he was not yet on the heart lung machine. This must be one strong heart, as somehow they help it beat again. This was the third "code".

Soon, the heart lung machine had taken over, circulating his blood. His heart does not beat,  but it is all right this time. The surgeon has begun to address the catastrophe inside his chest.

Any who may have been praying continued to pray.

The Outcome
It is too painful to wait longer to find out what happened to him.

He came though surgery beautifully. There was no brain damage. He went home in three days!

He is counted now among those amazing survivors who, against all odds, are still with us. Aortic dissection survivors and their families, like John and Tina Becerra,  may describe miracles and angels that helped save their lives. Surgeons such as Dr. Mark Poole who performed emergency surgery on a young mother   mention the limitations of human capability under these extreme conditions.

What Helped This Man Survive?
As they worked on him, some things became more clear about what had been happening inside.

A Tear in his "Heart Sack"
There was a lot of blood in his chest cavity. It appears to have leaked out from the sack that surrounds the heart, called the pericardium. When his heart stopped the first time, it was likely because the aorta ruptured open and blood flowed out and into this sack around the heart. When this happens, the blood compresses the heart and it cannot beat. This is called pericardial tamponade and is quite often the reason for sudden death during aortic dissection.

 But why did his heart start beating again? They found a tear in that pericardial sack around his heart, and blood flowed out through that tear into his chest. As the sack emptied of blood, the pressure around his heart lessened, and it could beat again. Remember the strong nurse that pushed so hard on his chest in his hospital room? It is likely those forceful compressions on a pericardial sack full of blood caused it to tear. It was one of the things that saved him.

Sealed by a Blood Clot
But how long could he live if he kept bleeding like that - from the aorta into the heart sack, from the heart sack out into his chest? What happened is that a blood clot formed over the ruptured spot in the aorta, sealing it and keeping his blood inside the artery.

In the OR, when they gave heparin, it likely dissolved the blood clot, and blood began to escape outside the aorta again. His heart stopped for the third time then.

His life was hanging by the thinnest of threads. Many things helped him survive in the midst of this disastrous aortic emergency:
  • He was not sent home from the hospital.
  • The ambulance did not come for him early.
  • A strong nurse pushed hard enough when doing CPR to break open the pericardial sack.
  • A blood clot formed over the rupture site in the aorta and stopped more blood from leaking out.
  • He was stable while moving from the cath lab to the OR.
  • A skilled aortic surgeon was nearby when calls for surgeons went out.
  • The surgical team restarted his heart from that last cardiac arrest and got him safely on the heart lung machine.
Why Not Find It Sooner? Let's Think of the Aorta in the Chest!
Why didn't someone think about this man's aorta? Why wasn't a CT scan ordered in the ER once a heart attack was ruled out? Aortic dissection is one of the three deadly causes of chest pain.

We know now that many hours went by before his heart stopped that first time. There was also a history of sudden death in his family. There was time to check his aorta. It was not done.

The medical literature indicates that there is a need to first of all think about the aorta. This paper from Germany mentions a campaign to help medical professionals think about aortic dissection in critical situations. This paper from Japan talks about the three conditions to rule out (including aortic dissection) when dealing with patients with chest pain, before turning to less immediately deadly causes.

We All Can Help Think of the Aorta
Greater awareness by individuals and families can help save lives. How? By learning about the aorta and any conditions that affect it, knowing and telling medical professionals about any sudden deaths of blood relatives, and reminding physicians and other medical professionals about the aorta.

Talking about the aorta before there are any symptoms may result in the earliest possible discovery. And if there are chest symptoms, it is vitally important to think of the aorta.

 If you have symptoms in your chest
 and are told by doctors it is not your heart,
 ask them to check your aorta.

Find It and Fix It First!
What does finding it first mean? Finding it as early as possible, before it has an opportunity to injure. If it has already injured, finding it before it has an opportunity to kill. When dealing with the aorta in the chest, early detection may save lives.

Searching online for "aortic dissection law firm", one law firm's website has a detailed description of aortic dissection and the risk factors associated with it. When physicians and medical professionals think of the aorta, particularly along with the other reasons for deadly chest pain, the aorta can be ruled in or out. When physicians do not think of the aorta,  lawyers seeking damages may follow.

This is a path that no one wants to travel. 
Let's find it and fix it first!

It all begins with thinking 
about the aorta,
Creating a Climate of Hope.