One of the most common, congenital heart valve disorders is a bicuspid aortic valve ^[1]. 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 ^[2] as the valve tissue degenerates (stenosis ^[3], regurgitation ^[4]).

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 ^[5].

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 ^[6] 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!