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