August heart check-up

I'm never sure what to share so I'll start at the beginning with Louis' most recent heart check-up.
Louis had an appointment, August 29th, for his routine echo, EKG, and over all heart wellness check. As always, Louis laid perfectly still for his echo with our favorite sonographer, Jordan (he looks just like Dan!). He also shared with us a 3D print of Louis' heart! So amazing to see and hold. Pediatric heart surgeons are doing some really incredible and unbelievable work.
Then we had a visit with his electrophysiologist who tested Louis' pacemaker and assessed how his heart is using the device and he made a few adjustments to his battery usage and sensitivity. Once Louis' cardiologist arrived I was prepared to ask questions about long term risks and complications, as well as make a request that we remain at 6 month check ups. I'm not ready for annual check ups and I prefer quarterly checks, so 6 months was my compromise.
She asked the usual questions about Louis' behavior; how's his appetite/sleep schedule, stamina, development, etc. and then she told us that Louis' heart function has declined about 20% since his last check up in April. She went on to describe that heart function is the squeeze of the heart, it's measured in a percentage of how much blood is squeezed through the heart at each beat. A normal heart functions between 50-70% and Louis' function is at 37% (57% in April). This is a very significant and concerning change.

We have spent the last two weeks adding and adjusting medications, going for tests, and scheduling some early intervention. Immediately, our first concerns are Louis' coronary arteries as he has had complications with them before (and his original heart condition required these arteries to be relocated). We also have concerns about his aorta, as it used to be his pulmonary valve and since his arterial switch operation, it has had some leaking.
Louis had a sedated CT scan last week and it showed us a little more, but it presented as expected. Which means, we believe the issue is not structural (meaning we aren't currently facing open heart surgery at this time). Instead we think Louis is presenting signs of Pacemaker-induced Cardiomyopathy. That's a hundred dollar term to explain that since the right side of Louis' heart is being paced (one lead to the top right atrium, one lead to the bottom right ventricle) that the left side of his heart has gotten lazy and isn't doing the squeezing job its suppose to. Because we aren't in an emergency situation...yet, we are going to get a consult from another hospital to get a few more expert eyes on the situation.

In the mean time, Louis' team of doctors and surgeons have met and decided the next step will be to perform a heart catheter (see this cool animated video for more information: https://www.webmd.com/heart-disease/video/cardiac-catheterization). They will create a small incision in his groin, and then a catheter will be led through the main vessel in his leg all he way up and into his heart. They will then be able to get a clearer view of his heart- paying close attention to his coronary arteries, checking the pressures of the heart, and temporarily pacing the left side of the heart.
If, by using temporary pacing wires, they're able to restore Louis' heart function, we will then schedule surgery to add an additional lead to his pacemaker.
All of this to say, we have certainly been through worse but we are currently experiencing unforeseen complications in Louis' continued heart care. I'm always struggling when people ask if Louis is all better. The hard truth is we will always have to be prepared for unexpected complications. His heart is really unique and though he has had a really wonderful year of positive growth and is exceeding developmental milestones, this is another growing pain. Louis' growing pains will never be able to exclude heart surgery or cardiac intervention.
This is where we are right now.

As always, much of this could change based on what they find through the heart catheter procedure. We have several appointments this week, including an in-patient hospital sleepover for his cath.
We would love to read your well-wishes and encouragements, if you please! Specifically, if you pray, we've been praying for healing from Fr Solanus (Blessed Franciscan monk from Detroit).
I've included some photos from our recent adventures. We took Louis to Manistee for his birthday (September 8th) and we spent the night on the SS Milwaukee, a retired train and passenger transport ship. It was very unique and fun! He got an adorable pedal firetruck for his birthday, and had his first experience on fair rides this past weekend.

Through all of this, I'm confident
he knows how loved he is. Keep us in your kindest thoughts.
I'll update as we learn more.

Much love,

Nicole, Dan, and Louis