Sunday, March 18, 2012

Sudden Death and Thoracic Aortic Disease - The Coroners' Cases

I have been privileged to hear great surgeons speak about treating aortic aneurysms in the chest. I have listened intently as they describe calls from the ER that must send adrenalin rushing through them. Someone has a torn (dissected) aorta and help is needed - now!

Only rarely, however, have I heard them speak about rupture. The reason is simple. Generally, these people are already dead.

I have also heard surgeons talk about replacing and repairing aortic valves. Yes, there is a great deal for them to describe, discuss, and even to debate. However, once again, I have rarely heard them speak about a certain group of individuals. These are people who "drop dead" suddenly - people who have aortic valve stenosis. This paper from Taiwan published in 2007, mentions that of known deaths from aortic stenosis, 20% of them are sudden.

These are the coroners' cases.

This week I spoke to a woman whose father, out fishing with a friend, took just three steps and dropped dead. It was just a week ago. He was 58 years old. Those of you from BAV families will recognize him - strong, healthy, never sick - but he also never knew he had a BAV. It was left to the coroner to advise the family, in no uncertain terms, that they should all be checked. Shocked and fearful, they are searching for understanding and support.  They want to be sure that this does not happen to anyone else in their family.

Sudden Death and Aortic Rupture
I remembered again the first time I spoke to Stacey Grieshop, years ago now, about the loss of her husband, Doug. Doug's death was such a shocking blow.  It was the coroner who had told Stacey "I don't know why your husband died" and proceeded with an autopsy. It was the coroner who found the reason, in Doug's aorta and in his heart - the break in his bulging aorta, and a bicuspid aortic valve in his heart. Doug's aorta had ruptured.

Sudden Death and Aortic Valve Stenosis
I also remembered a more recent conversation, just over a year ago, with a grief-stricken young widow. She had found her husband dead in their home. The autopsy showed that his aorta was completely intact. He had been diagnosed with bicuspid aortic valve stenosis just a few months earlier. How could something that seemed relatively benign at the time kill him without warning? He was 40 years old. His wife and their three young children were left alone, in the new home they had just purchased with such happiness.

Telling It All

Brent Warren
It is wonderful to tell about the happy outcomes, the victories over thoracic aortic disease - Bob Gies' return to biking and skiing. Derek Owens' return to basketball. Brent Warren playing baseball.

They have resumed exceptional, active lives following their surgeries. We are enriched and inspired by their lives. In their experiences, we find hope.

But we must tell all the stories, including those so distressing we cannot bear them. We do it to mourn them, to remember them. We do it to remind everyone that there must be a way to identify these people, to help them before they become a victim. We do it in hopes of saving others.

 Aortic Rupture at 19

"Volleyball court rebuilt to memorialize Duquesne U. student"  For anyone coming upon this headline, they might wonder what could have happened to such a young person. The reason is given in this moving Duquesne University account of family and friends gathered in the Duquesne Chapel,  Student remembered for zeal, humor . Many will recognize in Murph so many characteristics found in those with thoracic aortic disease  - an amazing athlete (as the article mentions "the fittest person on the team"), high energy, full of life. How can this happen?

A Voice for the Voiceless

Sudden death is indescribably horrific. There is no time to say goodbye, no time to save, no time to comfort, no time for just one last expression of love.

At the Bicuspid Aortic Foundation, we must be the voice of those so suddenly silenced by aortic disease. The voice for families and loved ones, who must live with the pain of their loss.

Yes, these are the coroners' cases. And sometimes, in addition to their tragic loss, when there is no autopsy, no understanding, no precautions, others in their families die too.

If you have suddenly lost a loved one, 
 you may save your own life and that of others
by understanding the cause of their deaths
and seeking expertise to help you.

You are not alone in your search. We are here to help you.

Arlys Velebir
Bicuspid Aortic Foundation

Sunday, March 4, 2012

Bicuspid Aortic Valve and A Grandmother's Heart

The following is a fictitious letter, based on the experience of a bicuspid aortic valve family. Only personal details have been changed to protect their identity. All names are fictional, and any resemblance to anyone living or dead is unintentional.

Dear Bicuspid Aortic Foundation,

I am one of those people with a bicuspid aortic valve that have never needed surgery of any kind. My health has been good, and I am now in my ninth decade of life. But I have to tell you that to my great sorrow, I lost many of my family members to sudden death, some of them at a young age. Two things seem to run in our family: "heart trouble" and aneurysms. All of my brothers and sisters died from just these two things, and I am the last one left.

My brothers and sisters and I were all quite young when we lost our older brother, Tom - the first sudden death in our family. He was always so healthy. One day, he just "dropped dead". They said it was a heart attack. He was only 36 years old. Somehow his wife and little ones made it through those hard years without him. It is a long time ago now, but remembering still hurts. Children need their father.

I had two other brothers who lived longer, Peter and John. When they died, John at 66 and Peter at 74, we were told they had heart attacks, too. Then my sister, Mary, died at age 64. It was sudden too, but this time we knew it was not a heart attack - they told us that Mary had an aneurysm in her chest.

After so much sudden death in our family, I was so glad to still have my sister Jane. She died later -  suddenly - of a brain aneurysm.

Today I heard that one of my grandsons has a heart like mine. He is a healthy young man, athletic, and the father of three of the cutest little boys - my great grandsons. Lately though, he had some pain in his chest while exercising. He went to the doctor because his voice became strangely hoarse, and sometimes he seems to have trouble swallowing his food. The doctor heard a murmur, and that is how they found he has a bicuspid aortic valve. They told him it is leaking - I hope it is not too bad. Thinking back, I remember now, too, that my granddaughter, Sarah, one of his sisters, was told that she had a heart murmur when she was a little girl.

 I have not said this to anyone in the family, but I am so afraid for my grandchildren, and even my great grandchildren. I have lived many years with a bicuspid aortic valve - but so many in our family have died these terrible, sudden deaths.

My grandson, Andrew, is going to see a special heart doctor soon. He is about the same age as my brother Tom was when he died suddenly. Surely, they must understand more by now. Surely they can help him. Just like it was back then, today there are children - three little boys - who need their Daddy very much.

Do you think that some day there will be answers for families like ours, with "bad hearts" and aneurysms? I am praying that day will come soon. I am old now, and I know that I may not live to see it. Please remember my family, and others like us everywhere, when you meet someone with a bicuspid aortic valve. Some of us are blessed with good health, but our lives are shadowed, our hearts broken, with sorrow and grief when we suddenly lose those we love so much.

Yours very sincerely,
Andrew's Grandma

Post Script:

In this one family, two sisters died of aneurysm - one of the brain, one in the chest. Three brothers died suddenly of supposed heart attacks, not verified by autopsies. One sister remains alive in her 90's, with a known bicuspid aortic valve. This woman's grandson, an active, athletic man in the prime of his life, has been  diagnosed with  a leaking BAV, and was symptomatic in his chest with exercise, along with other symptoms that have been attributed to GERD.

It is well to remember that for every person with a bicuspid aortic valve that may live a long lifespan, there may be others in that same family who will suffer very different fates.

May we be moved to find answers,
for this Grandmother and her family
and for other families everywhere, 
creating a climate of hope.

Arlys Velebir
Bicuspid Aortic Foundation