A Personal Update

On the day I was born, I was diagnosed with a congenital heart defect known as Aortic Valve Stenosis. Essentially, due to a typo in my genetic code, one of the valves in my heart which is supposed to have three flaps, only has two.

Bic

Those two flaps leave a large gap in the valve, and allow a significant amount of blood to leak back into the heart after it beats (known as valvular regurgitation). This means that my heart has to work much, much harder than yours to pump the same amount of blood – and therefore oxygen, through my body.

 

The extra strain on the heart due to a bicuspid valve eventually leads to two complications, which have only become problematic for me within the last year or so. First, the valve itself has become calcified.  Literally, the extra stress on the valve has caused calcium deposits to build up in and around it.  From the Mayo Clinic:

 

“With age, heart valves may accumulate deposits of calcium (aortic valve calcification). Calcium is a mineral found in your blood. As blood repeatedly flows over the aortic valve, deposits of calcium can accumulate on the valve’s leaflets. In some people — particularly those with a bicuspid aortic valve — calcium deposits result in stiffening of the leaflets of the valve. This stiffening narrows the aortic valve.”

 

The second complication is enlarging of the heart, due to the extra work it’s being forced to do.  From the National Institutes of Health:

 

“Valvular regurgitation forces the heart to work harder to transport blood. Over time, the heart muscles expand to compensate for regurgitation, leading to an enlarged heart. An enlarged heart deforms the heart chambers and causes further damage to the heart valve, increasing the severity of valvular regurgitation.”

 

An enlarged heart not only increases the risk of heart attack and stroke, but also arrhythmia and bacterial infection in the lining of the heart (endocarditis).

 

This genetic disorder is not as rare as you may think. About 1–2% of the population have bicuspid aortic valves, but the condition is twice as common in men.

For most of my life, this defect meant nothing more to me than a funny-sounding heartbeat, which was a hit with med students during doctor’s visits.

 

Normal Heartbeat: 

Aortic Valve Stenosis Heartbeat:

 

However, in recent months, I have noticed the emergence of classic cardiac symptoms. Specifically, loss of stamina, shortness of breath, and chest pain. I scheduled an appointment with a cardiologist, who I’d never met before, since I’m still new to the area. He recommended three tests: an echocardiogram, an electrocardiogram (EKG), and a chest x-ray to determine whether my condition had worsened.

 

Normal EKG: 

Normal

 

My EKG: 

Avsdekg


My Chest X-Rays:

Sam_0105-1Sam_0108-1Sam_0109Sam_0110-1Sam_0111-1Sam_0112-1

 

The EKG and x-ray were quick and easy, but the echo was more involved.  As I’m lying in the dark exam room, I’m able to watch the screens as the test is performed, and I can basically figure out how the test works (it uses the Doppler Effect to determine whether blood is flowing backwards through the valve), but not what the results are. And of course the tech won’t comment on what’s going on. I’ll have to wait for the doc himself to tell me what they found. 

Echo

 

It turns out, over the past year or two, since my last cardiac check-up, my condition has worsened significantly.  To determine the extent of the damage, what steps need to be taken to correct it, and how urgent that is, they needed to collect more data, this time from inside my heart.  This is done through a procedure called cardiac catheterization.

 

On Thursday, I went in to have this test performed.  After being prepped, stabbed, and drugged, I was brought into a full operating room. Then, I was drugged again. Woo.  While conscious, but completely out of it, the doctor made an incision in my femoral artery (in the upper thigh)  He inserted a catheter into that artery, and ran it all the way up into my heart. They used pressure sensors, contrast dye, and x-rays to gather data on my heart function.

 

The results weren’t good. The short version is, I’ll be returning home to Boston within the next month to undergo valve replacement surgery. This is major open-heart surgery, and the recovery process is daunting.  The type of procedure chosen by the surgeons at Children’s Hospital will affect the length and severity of my recovery. There are pros and cons to each procedure, which I may go into another day.

 

This could happen any day now, and when it does, you probably won’t hear from me for quite a while. But if any of you reading this are in the Boston area, and already know how to contact me, I’ll be desperate for social interaction while I’m reovering.  For those of you too far away to visit, I urge you to consider becoming an organ donor.  The need is very real, for me and more than 100,000 people like me.

 

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