Pretty much ever since my heart surgery in 1984, I’ve resigned myself to a kind of semi-permanent suspense. Would I need another surgery? Am I “fixed” now? What would life be like now that I could play outside and have a reasonable expectation of not passing out? How long would that last?
A common refrain from those in the adult community of congenital heart defect survivors is “You are never fixed.” While I was still working in the Quad Cities in the early 2000s, something prompted me to retrieve all of the records that may still remain from my heart surgery. I contacted Seattle Children’s Orthopedic Hospital and after signing a release they mailed me, received a hefty 100+ pages of every heart visit going back to my actual birth. I wrote a bit about what I found there back in 2006.
I had also started hanging out in the Adult Congenital Heart Association forums where I learned that I wasn’t really fixed. I knew that of course, but acknowledging it is another thing. But how “not fixed” was I, exactly? Thus I’ve been seeing one Adult Congenital Heart specialist or another since that day to make sure I was never surprised.
After years of getting an essentially clean bill of health for someone with an abnormal lump of muscle vaguely resembling a functional heart, I got my first warning in 2019. Sure, my ejection fraction has always been bad, there’s always that murmur they hear, and my right bundle branch block may eventually graduate to a full heart block where I’ll need a pacemaker. But those were all known elements. What could they add onto the list that would concern me?
Aside from the yearly Echocardiogram, I get an MRI roughly every 5 years so they can watch a contrast material flow through my heart. The latest one in 2019 showed that my aortic root was dilated to 4.9cm. I didn’t know this was very serious at the time, because my cardiologist’s nurse said something to the effect of, “The doctor would like to get another MRI because it looked like your aortic root was a little dilated.”
It didn’t help that much had also changed fairly recently. This was shortly after the time I joined 2ndQuadrant in 2017. After we moved to a town totally isolated from society by 90 minutes of driving on all sides. After I switched to much worse health insurance plan offered on HealthCare.gov since I was no longer covered by my previous employer, meaning that the MRI wasn’t covered by insurance. So travel was much more inconvenient, I was still paying off the previous MRI, and I was more than a little ticked off at Northwestern Hospital for refusing to negotiate the payoff amount. Besides, it was only “a little” dilated, right?
So I let the inconvenience and lack of urgency win out and slid into a bit of complacency regarding my heart. What changed that was my rude encounter with Peripheral Nerve Hyperexcitability in late 2020. I’ve experienced a greatly increased amount of Premature Ventricular Contractions since then, and if not for the badly timed Covid pandemic, I would have immediately rushed to a Cardiologist in Peoria.
Now that Covid hysteria has died down for the most part, I finally made that appointment. The ongoing PVCs are annoying and mildly unnerving, and there was always that dilation to check out. So I made an appointment at the Congenital Heart Center at Peoria’s OSF Healthcare. After getting a reference from my GP that is, as they don’t take direct appointments. I’ve never had that problem previously, but I knew they’d want to see me one way or another given my history.
And so on the first week of January this year, I got my first Echocardiogram since 2018. The cardiologist saw me right after the echo so we could go over the results and some of my other history. So I told him about the nearly constant PVCs, and we loosely discussed the merits of beta blockers. My heart rate isn’t nearly as low as it was during my DDR days, but a resting rate of 52 is still on the lower end, and it was 47 when they took my blood pressure. Still, he prescribed a 24-hour Holter monitor to see just how bad the PVCs were, and if my heart rate was “too low” for a beta blocker.
Then he told me that he wanted to schedule an MRI to evaluate the aortic root dilation they saw on the echo. Wait a minute, I’ve heard that refrain before! I don’t think I asked about it then, but I nodded along, knowing that I was overdue for that anyway, and went home. A day or two later, the writeup for the echo showed up on the online health portal, so I took a look and saw that my dilation was 4.64cm. Curious, I examined the results from Northwestern, and they said it was 4.9cm. Well, it went down, but echos aren’t as accurate as MRIs, so how much is too much?
Down the rabbit hole of aortic root dilation I went, since my previous cardiologist never really brought it up before my scan in 2019. It turns out that 5.0cm is usually when they recommend surgery. What? Why did nobody say anything before 2019? So I checked all of my previous echos and came up with this:
- 2008 - 3.3cm
- 2010 - 3.7cm
- 2012 - 3.4cm
- 2015 - 3.8cm
- 2016 - 3.9cm
- 2019 - 4.9cm
- 2023 - 4.64cm
All of the missing readings are from echos where the measurement was either omitted, or there was a note suggesting they had trouble getting visibility of my aortic valve. So my aortic root was steadily increasing basically since the first time I saw them 15 years ago. But 3.3cm itself is abnormal since the mean size of an aortic root for males is 2.1cm. Assuming 1-2mm of dilation every year, mine has been above normal since I was a teenager, and probably earlier.
And that’s when it really clicked. I was never fixed. They did what they could, but congenital surgeries are still in their relative infancy, especially for children. The earliest of these were performed back in the 60s, so I’m part of the 2nd generation of childhood heart patients. Even if they found and repaired everything they could think of or physically see, nobody really knows the long-term implications or possible complications. Even if they missed nothing, I have dextrocardia, so the arteries and vessels are completely and unpredictably divergent.
Imagine a garden hose with a kink because it is twisted or pinched off. If the hose were weak enough, pressure would build up before the kink until it threatened to burst. Arteries are fairly robust, but they’re not infinitely flexible. My heart is twisted around and lays more to the right than the left. It wouldn’t take much, perhaps a few millimeters of narrowing at just the wrong place, and the pressure at the base of my aorta would slowly cause the root to dilate year after year until the pace accelerated.
That’s what happened some time between 2016 and 2019. My personal theory is that my gallbladder surgery in 2016 put a lot of extra stress on my system in general, and that caused the jump from 3.9cm to 4.9cm. But it’s fairly clear from the trend line that a second heart surgery is inevitable. Either now because 4.64cm was simply Echocardiogram variance and I’m still at 4.9cm or higher, or because my anatomy effectively guarantees future dilation.
Why had my cardiologist said nothing previously? Why did it take the jump from 3.9cm to 4.9sm to cause concern, when my aortic root was already at roughly twice the normal diameter and had been steadily expanding since at least 2008? I was expecting a potential valve replacement due to my lingering murmur, or maybe a pacemaker due to the scar tissue from my previous surgery. This came completely out of left field.
Meanwhile, the results for the Holter came back as well, showing a 4% average PVC load over the 24-hour measurement period. They tend to come in batches, where hours will elapse with none or very few, and times when they feel almost constant. So my new cardiologist called in a low (25mg) dose of metoprolol succinate, and told me to take half a pill every day to try to control the PVCs. I still haven’t worked up the courage to do so given my fairly low heart rate. I’ve been doing research instead, trying to figure out how much heart rate reduction I can expect, and weighing that against my urgency to reduce the PVCs.
The upcoming MRI in February will likely be the deciding factor on whether I can expect a second heart surgery in my near future. If the dilation really has somehow receded since 2019, maybe I can expect further good news in my yearly followups from now on. If not, I need to get comfortable with the fact I’ll need another surgery due to my complicated anatomy, even if it’s not the one I expected. It is the other proverbial shoe.