“My Heart Condition”

**UPDATED – to include additional information about my first two surgeries**

I’ve used this phrase a lot over the years and I thought some might be curious (or scientifically / medically minded) to know what it is I mean by this.

Heart Defects

First and foremost, my heart condition is a “Congenital Heart Defect” (CHD). Did you know that heart defects are the number 1 birth defect? Just about 1 in 100 babies are born with one.  This can be anything from a murmur, or valve issue, to PDA’s not closing in the proper time, to much more complicated – multiple issue formation problems.  Another item to note is that for those of us with a CHD – we have a slightly higher chance of our children being born with a CHD – about 4-6% (instead of the normal 1%).

My CHD

So what exactly is “my heart condition”?  It’s called pulmonary atresia, which is a complete blockage of the pulmonary valve. In my case, the pulmonary valve never formed at all – there was just a wall of heart muscle where the valve should have been.

When a baby is in utero, the baby is not breathing (or using his or her lungs), so there are some “short cuts” in the heart that allow for blood flow. The PDA & the PFO are small holes that allow a baby to pump blood while bypassing the lungs.  Upon birth, normally these holes close (if they don’t as mentioned above, it’s a heart defect – usually fixed by medicine or if needed, a fairly simple medical procedure).

In my case, the holes closed as they should, but in doing so, it took away the short cuts and prevented blood from making it to the lungs to pick up oxygen. About 12 hours after birth, I “crashed” – that is I started turning blue, also called cyanotic. I was whisked away from my suburban Chicago hospital and shipped via ambulance downtown to a large children’s hospital.  There they did my first heart surgery while I was just about 24 hours old.

The Fix

I don’t have all the details, but basically as I understand it, they put in a Waterston Shunt (grafting 2 vessels to reroute the blood).  At 30 days old, they did a second surgery, basically “poking a hole” where the valve should have been to increase my oxidation levels.  And by 40 days, I was on my way home. Both of these surgery are called “Open Chest” because the heart was not stopped to do the surgery, like with open heart surgery, and a bypass machine was not used. (Side note, if this happened today – there is now medicine to keep the PDA open or an increased chance they would catch the issue via sonogram- so they can buy time and come up with a plan. )

Obviously, I don’t remember, but I’ve heard the story so many times – it was pretty dire.  My dad overheard the surgeon arguing on the phone about getting an Operating Room “stat – or this girl is going to die”. Apparently, there was not an OR available right away. Yikes. I can’t imagine hearing that.

Last Surgery

Then when I was bout 2 and a half, I did have Open Heart Surgery (I’m part of the ‘zipper club!”). I believe this surgery was to repair a small hole called a ventricular septal defect (VSD), which is a slight hole between the right and left ventricle wall. And that was my last heart surgery – over 30 years ago.

Through the Years

What all this means is that through the years, I’ve had to go to the cardiologist almost every year, just for check ups and to keep an eye on the size of my heart. Because I do not yet have a pulmonary valve, I have no “gate keeper” regulating blood flow. This, in turn, means some blood goes backwards (called regurgitation), and thus, my heart is less efficient. Through the last 32 years my heart is slightly enlarged – and overall less efficient than a healthy person’s heart.

Another thing that can’t be left out is that I was considered one of the worst cases every to eventually do so well. If I had been born just a few years earlier, or in North Dakota (where my parents lived until my mom was 7 months pregnant & they moved to Chicago), I would not have survived. Because of this, I’m kind of on the “cutting edge” of Adults with Congenital Heart Defects. There aren’t that many of us with complicated CHD’s that are now alive and thriving in adulthood.  In fact, because it’s such a new specialty field, I had to go to the children’s hospital until I was 23 when they finally completed setting up a transition team for specialists at the University of Washington. I often joke that they should pay me for all my tests as I’m unique and they are learning from me.

When I moved for college, and then after college to Texas, it presented a major priority to find a new cardiologist.  **Pet Peeve Alert** So many individuals hear I have a heart condition and recommend a cardiologist when they hear I need one. I know they are trying to help and have the best intentions – but I can’t go see just a regular cardiologist. I don’t have heart disease, I’m not at risk for a heart attack, I don’t have clogged arteries. Sorry – regular cardiologists have only read about people like me in text books. 🙂

I digress. So what does this mean long term?

Long Term Outlook

Well for starters, I still do not have a valve. So my less efficient heart is often noticeable to me in the fact that I have less stamina than most 32 year old women.

It is also very common for me to have irregular heart beats and palpitations. Most of the time this is normal – though a few times through the years, I’ve noticed them more frequently which I report to the doctor and usually end up having a heart monitor for a day.

Because my heart is enlarged, and will probably continue to become more enlarged over time, it is key that I go to the doctor every year so they can keep track of how big it is, and how much ‘regurgitation’ is taking place. At some point, my heart will reach a threshold of irreversible damage. And should this happen, or should it completely wear out, the ultimate outcome is “Congestive Heart Failure.”

The goal is that by keeping track of where it at, I will have a valve put in before it reaches that threshold. The reason we wait – is because 1. Medial technology is always improving and 2. Once I’m done having children, I’ll have more options available.

They would put in either a live tissue valve (which believe it or not, is done in a way that anti-rejection meds are not needed) or a mechanical valve (which requires blood thinners and would not be safe for a baby if I were pregnant).

So we wait.  8 years ago, I was told “best case scenario, I’d probably need a valve in the next 10 years”. Last year, I was told “”best case scenario, I’d probably need a valve in the next 10 years”. So I gain some time!

There you go. If you ever wondered what I meant when I said “my heart condition,” now you know.

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2 Comments

Filed under cardiology, health

2 responses to ““My Heart Condition”

  1. Hi! Just wanted to say thank you for this post. I too am a member of the zipper club having had two open heart surgeries as an infant (Fallot’s Tetralogy). I too am one of those kids who somehow turned out to be just like everybody else and lead a pretty ace life. After my last surgery at two and a half years old, my parents were informed that “best case scenario” was surgery again in five years – tops… If I made it that far. I am yet to have surgery again and I am nearly twenty-nine! I have been terribly remiss in following up with my cardiologist and as I am seeing him next week, I am getting a little nervous about something that rarely crosses my mind. Your post reminds me that I’m not alone in my situation, so yeah, thanks. It’s not something I talk about often either because I never want people to think it’s a sob story – actually it’s the opposite!

    And end ramble haha 🙂

    • Thanks for taking time to share your story!
      That’s so terrific that you are doing so well. Isn’t funny how a lot of times it’s educated guesses and estimates, but we hold on to them as “that’s the way it is”? And then – it’s a little, or a LOT, off.

      I hope your appointment went well.
      I know I went a period of time a few years back where between college and lack of insurance, I went longer than I wanted between doctor appointments. I too was nervous on my return trip.

      And I totally hear you about it not being a sob story. I find that it can be hard work balancing between being honest with people when sharing my story and not treating it as a pity party. I can’t keep up 100% with people my age and I need them understand not to expect me to, but I also don’t want them feeling sorry for me. Both of us are quite fortunate. Considering how things could have gone, we have wonderful lives.

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