Unicuspid Aortic Valve… Really?
One of the most common, congenital heart valve disorders is a bicuspid aortic valve. Patients born with a bicuspid aortic valve have only two leaflets. Normal aortic valves have three leaflets. Ultimately, this can significantly compromise blood flow through the heart and body as the valve tissue degenerates (stenosis, regurgitation).
Earlier today, I received an email from Nikki. I perked up in my chair as I read her email about a unicuspid aortic heart valve.
Nikki writes to me, “Hi Adam - I am trying to find out more about my 2-year old heart’s condition when I came across your site. He has been diagnosed to have a unicuspid aortic heart valve. At what age will this start to effect him? Do you have any additional info on this? Greetings from Cape Town, South Africa! Nikki (Mom)”
You can see what a unicuspid aortic valve looks like in the pictures above. Notice that there is a single cusp which has been severed at the top of the valve. Both of these removed valves were severely calcified.
Unicuspid heart valves are very, very, very rare. In my research, I did not locate any clinical datasets which tracked the disorder and its impact upon patients (by age, by sex, etc.). However, I did find a few, surgical case studies of aortic valve replacement patients at 26 and 52 years of life. That said, it appears that this disorder can trigger heart valve disease at different times in the patient’s life.
I hope this helps explain more about unicuspid aortic valves.
Keep on tickin!
About The Author: Adam Pick is a double, heart valve surgery patient and author of The Patient’s Guide To Heart Valve Surgery, a unique book which integrates the clinical facts of heart valve surgery with the personal experiences of 78 former valve surgery patients. To learn more about Adam and his heart valve surgery book, click here.
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December 18th, 2008 at 10:58 pm
Dear Adam,
I just had a aortic valve replacement on Nov 17,2008 with a 4 cusp or leaflet valve. That too is quite unusual. One was not functioning at all and so there was severe regurgitation back into the ventricle. My surgeon said I was born with this. Interesting heh. Jean
December 19th, 2008 at 9:30 pm
I had aortic valve replacement 5 weeks ago at the Cleveland Clinic. What they suspected was a bicuspid valve actually turned out to be unicuspid. I didn’t realize that was so rare.
December 21st, 2008 at 1:02 pm
I have had three pleural effusions since my aortic replacement on Oct 30th.
The procedure to drain them is unpleasant and each episode seems to take more energy out of me. Do you have any info on this, how long they usually continue and if ther are nay more permanent solutions? Thanks
February 24th, 2009 at 9:36 pm
DEAR ADAM, I JUST UNDERWENT WHAT I THOUGHT WAS A BICUSPID VALVE REPLACEMENT. DURING SURGERY IT WAS IDENTIFIED AS A UNICUSPID. I AM 40 YRS OLD HAVE THREE CHILDRED AND LIVE A ACTIVE LIFE. I WANTED TO LET NIKKI KNOW THAT AS LONG AS YOU WATCH IT EVERY YEAR WITH A CARDIOLOGIST HER CHILD WILL LIVE A GOOD LIFE
June 1st, 2009 at 8:31 pm
Hello Everyone –
I am a 30 year old Male who was born with a unicuspid aoric valve. I had surgery at age 14 at Childrens Hospital of Milwaukee at which point they cleaned up the valve and split it in half (it is now bi-cuspid). I had a second operation at age 25 at Cleveland Clinic where they replaced my Ascending Aorta and again cleaned up the Aortic valve (still bi-cuspid). I have a son that is 11 months old with a clean bill of cardiac health!
Nikki … I played Varsity Soccer, Varisty Tennis, Swimming, and Basketball. Your son will be fine, but trust me he will have a different appreciation for life then you can imagine!
I am currently only on a Beta-Blocker and low dose aspirin. If you have any questions feel free to contact me at ko37784@gmail.com
Thanks.
Scott H
July 16th, 2009 at 2:08 pm
Hi Nikki and Adam,
I am the parent of a 17 year old son. He was diagnosed with bicuspid aortic stenosis when he was one year old. We were told he probably wouldn’t need surgery until he was 30 or 40. He was fine with a normal childhood until he turned 15, when he started having rapid heart beat. I knew then things were changing for him. When he was 16, he started having chest pains. We were told he needed surgery soon. He had open heart surgery one week later. Last August, at age 16, he underwent aortic valve replacement surgery in which he received an artificial valve. I chose the artificial valve hoping he would not need another surgery later in life. During the operation they found he actually was unicuspid, but the way the valve moved on the echocardiogram it looked as though it was bicuspid. The surgeon said we were lucky we chose not to wait awhile for the surgery. My son recovered quickly from surgery and is doing great. He is a typical high school student and will be a senior this year. I just wanted to let you know that it’s been almost a year since my son’s surgery and he has a great, healthy future ahead of him. Good luck with you and your son.