Saturday, February 7, 2015

The Heart of a Man - Angelo's BAV Diagnosis

"I am writing to you because I simply don’t know where else to turn." 

On any given day, somewhere in the world, a man born with a bicuspid aortic valve learns about it for the first time. 

Why a man? Because BAV is up to 3 times more common in men than women. What is it like for this man, typically healthy, with little need for visits to doctors' offices? 

Angelo and his wife, Australians,  visited with some of us in southern California
Arlys Velebir, Mary Houchin, Richard Houchin, Angelo, Marie, Dr. Sharo Raissi
Here we share one man's experience. Angelo Doukas has given us permission to share the letters he wrote to the Bicuspid Aortic Foundation shortly after his diagnosis. We have removed a few personal details and added subtitles, but the following, mainly untouched,  is Angelo's beautifully written account of his experience .

First Ever, Routine Heart Check

Just over one week ago, a routine heart check (the first I have ever done in my life) revealed that I have a bicuspid aortic valve and aneurysm of the ascending aorta.

My cardiologist, who I had just met, explained what this meant and showed me the ultrasound images.  He explained that a normal aorta is about 3.5 cm and that when my aneurism grows from its current 4.7 cm to 5 cm, surgery will be required.  He explained how the aortic valve is supposed to operate and how mine does, how the condition has familial links, and so on.  He asked to see me again in six months.
I think I will always remember his parting words for the rest of my life:  “nothing will happen to you in the immediate future, but it’s a problem”.  I’m not used to hearing those words.


I sat in my car afterwards in a daze, not quite sure what this was all about but knowing that it was serious.  I was in shock, but not yet emotional because I didn’t know enough about the condition to form any opinions.  

I came home, started researching on the internet and came across your website.  I read about the condition itself (I still can’t bring myself to use the term “disease”) and then read your stories of hope.  That’s when I became emotional. 

In a single day, my life completely changed.  Until that day, I was a healthy 55 year old who always looked after himself.  I am a vegan, I exercise, have never smoked, have very little stress in my life and a wonderful, supportive family.  My parents are still alive and well, and I looked forward to a healthy old age.

My Life Will Go On!

One week later, I am still emotional, but my focus has changed.  Your website and a follow up discussion with my family doctor have reassured me that my condition does not mean an end to my life.  It will never be the same, but it will not end.  If nothing else, I’m grateful that I’ve reached this age without symptoms and without requiring surgery.  I’m also grateful I discovered the condition at 55 years of age.  I have enough time to mentally prepare for the challenges ahead, and I have lived my life until this point in time without the burden of knowing what lay ahead!     

A Father's Heart

But as I said, my focus has changed.  This week I’ve been thinking of my two children, both of whom are in their late twenties.  My cardiologist (I am also struggling to say “my cardiologist”) has strongly recommended that my children are checked.  I know this is the right thing to do and have told them they should do it.  But I live in fear that one or both of them have this condition.  Just the thought of them having to undergo a future procedure is enough to weaken me at the knees. 

When I broke the news to my family doctor he looked me in the eye and said “I would much rather be telling you that you had this condition rather than cancer,  or a neurological condition, or an autoimmune disease”.  His words were comforting at the time, and remain so, but I can’t help but reflect on the fact that none of these conditions necessarily involves the rest of the family.

You have mentioned that one of the worst things about BAV is that it’s a silent killer because healthy people are blissfully unaware they have it.  I would argue that the worst thing is the familial link because of the guilt and emotions and repercussions that result from it. Your web site talks a lot about frail aortas, but right now I am feeling so emotionally frail thinking about my children that I often break into tears.

Going Back for More Information

Now that I am much better informed, I have booked another appointment with my cardiologist for early next week (there was no way I was going to wait six months!) to have some of my questions answered.   I will also take my wife with me, because I would like her to hear everything first hand.

After the Second Cardiologist Visit 

 He (cardiologist) had asked me to come back in six months’ time, so he was very surprised to see me.  I had booked the appointment without him knowing!  I think he was genuinely concerned that he had created so much anxiety in me, so he gave me as much time as I wanted.

I feel so much better after this second visit, for two reasons.  Firstly, it appears that my bicuspid aortic valve is in excellent shape and, at 55 years of age, is still performing as well as a normal tricuspid valve (no abnormal leakage, etc.) and could continue to do so indefinitely.  My wife can verify his exact words: “if the rest of your body was up to it, you could compete in the Olympic Games”.

Angelo chatting with Dr. Sharo Raissi
in southern California
 Although I have no intention or desire to compete in the Olympic Games, it was music to my ears.  He reminded me that he had made that point in our first session and, frankly, I believe he had.  But so many arrows were being thrown at me from all directions that I was only hearing the bad news.

Secondly, my aneurysm is 4.6 cm, not 4.7 cm.  Not a big difference, but I’ll take every millimetre I can get.  He mentioned that he had many patients (one of them was actually in the waiting room) who stayed on this size for many years.

In summary, your lovely letter, in combination with this second visit, has put me in a much better state of mind.  My fear about requiring imminent surgery has subsided, and I am more confident that surgery, at least initially, would only be required for the aneurysm and not the valve.

Believe me, I am not being complacent, nor am I taking anything for granted.  But as you so correctly pointed out, at least now I know a little more about what’s inside me, and I can work with my cardiologist to monitor the problem and act on it at the appropriate time.

My only remaining concern relates to my children.  I have advised both of them to have a test and, at least for the foreseeable future, I will now leave it up to them.

Thank you so much, Angelo, for allowing us to share this. I am sure it will help many  who are learning for the first time that they were born with BAV. 

                                                                                 Best wishes,
                                                                                 Arlys Velebir
                                                                                 BAF President