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We spent a good while today talking with the cath doctor, who’s also the attending on service right now.

One thing they’re weighing is whether to open up the conduit that carries blood from the right ventricle out toward his lungs. It started at 14 mm back during his first big surgery, and over the years it’s narrowed a lot as he’s grown. The idea would be to go in through a catheter and either stent it open or place an artificial valve there.

The hesitation is that it could backfire. Because his tricuspid valve is leaking so badly, opening that pathway up might just send even more blood flowing the wrong direction. An artificial valve also carries its own risks, and it’s a big piece of hardware for a kid his size.

On the better-news side, they all keep saying how great Isaac looks. They recently restarted his Lasix. It had been paused while he was fighting that virus, and now that he’s back on it the weight is coming down and the puffiness is going with it.

We track everything going in and everything coming out, plus his weight, to see how hard the Lasix is working and to make sure they don’t tip him too far the other way and dry him out. We’ve learned to keep our own log alongside theirs. With as many nurses and rotating teams as you go through in here, our numbers have caught real discrepancies in his ins and outs more than once.

The best part of the day had nothing to do with any of that. We spent most of it building a very cool Lego race car set (thank you, Anselma and Kevin!) and playing board games. He’s in great spirits.

Yesterday we talked with the surgeon again, and also met with one of the transplant doctors and a few members of the transplant team. By the end of those conversations, everyone was in agreement: transplant is the best path forward for Isaac.

The surgical repair is still technically possible, but the team walked us through what it would actually mean. To fix the tricuspid valve, they’d have to cut deeper into his heart than they ever have before. And if that surgery doesn’t go the way they need it to, there’s no good fallback. He’d go on ECMO and wait for a donor heart, and there’s a very real chance a heart wouldn’t become available in time. The surgeon put it plainly: going into that repair would be putting all our eggs in one basket. He said he wouldn’t be able to sleep at night doing it.

So we’re moving forward with the transplant evaluation. The team already believes Isaac is a strong candidate. It’s a process, there are steps and assessments involved, but we could realistically be on the transplant list as soon as next week.

From there, the big question is whether we wait as inpatients here at the hospital, where a heart could possibly come in the next six weeks to a few months, or whether Isaac is stable enough to go home and wait as an outpatient, which could mean somewhere between six months and a year or more. That’s still being figured out.

In the meantime, they want to try something next week, likely Wednesday. The tricuspid valve is leaking so severely back into the upper chamber that blood isn’t flowing the way it should through the baffles, and there are some leaks and obstructions in there too. The plan is to balloon and stent the main baffle to try to improve blood flow and take some pressure off the leaking valve and ventricle. It won’t fix anything permanently, but it may buy more time.

On a much happier note, everyone keeps remarking on how great Isaac looks. He’s been up playing board games and just hanging out with us on the couch most of the day. We’ve talked with him a little about the bright side of all this. He loves the idea that one day he might not need a pacemaker and could finally jump on a trampoline. He’s been retelling all of it to his sister, the whole plan, in his own words. He’s the sweetest kid.

Last night was the surgical conference, where all the cardiologists and surgeons sit down together with everything, the cath data, the echoes, the rhythm events, and hash out a recommendation.

The doctors came to talk with us around 11am. Their recommendation was to move forward with a heart transplant. With his complex anatomy and the repeat issues, they believed the best option is to start the transplant process now, before he worsens.

Then, about an hour later, the main doctor came back. The top cardiac surgeon, the same surgeon who did Isaac’s first surgery, hadn’t been able to attend the conference because he was in a long operation. And he was not so sure transplant was the best option. He believed there’s a path forward keeping Isaac’s own heart, and came to talk with us a few minutes later.

The way he sees it: Isaac’s heart has a great squeeze. The muscle itself is strong. The flow problems and the leaks, the narrowed conduit, the leaking patch, the leaky valve, are the likely culprits causing everything, and those are things he can repair. He wanted another day to talk with the wider team and build a real consensus, and he’ll come back to us tomorrow with a solid recommendation. If we move forward with the repair operation, it could happen as soon as early next week.

The repair would be another very big open heart operation with its own risks and its own long recovery road. And while transplant success rates are genuinely good, the body never stops trying to reject a transplanted heart, so there’s a countdown built into it. They don’t last a lifetime.

Tomorrow we should know which road they’re suggesting. Praying for wisdom.

Mom called me at 6am. I could hear Isaac screaming in the background, and she was sobbing that I needed to get to the hospital immediately. Isaac’s heart was beating way too fast and then the doctors had started chest compressions.

Here’s what happened, as I learned once I got there. Isaac grabbed his chest and screamed in pain, and Mom watched his skin go gray and his hands and feet turn blue as the staff ran into the room. His oxygen dropped into the 40s and they couldn’t find a pulse, so they started compressions. Thank God it was brief, about thirty seconds before they got a pulse back. He had gone into a lethal arrhythmia, a flutter like the ones before, but much faster. His top chambers were racing around 240 to 250 beats a minute.

What made this one so dangerous comes down to his pacemaker settings, and they figured this part out quickly. His pacemaker listens to the top chambers and paces the bottom chambers to match, and its maximum was set at 180. So when the flutter took off, the pacemaker chased it and drove his heart to around 170 to 180, so fast that his heart was squeezing before it could fill with blood. A dry squeeze like that is extremely painful, and it doesn’t move blood.

They got him out of the lethal speed and gave him sedating medicine to ease him, though he stayed in a slower flutter. They moved him from the inpatient wing down to the cardiac ICU, supported his blood pressure with medication, and that afternoon they cardioverted him again, and tripped him back into a normal paced rhythm. They also reprogrammed his pacemaker so it now maxes out at 140 instead of 180, so it can never chase a flutter that high again, and they increased his rhythm medicine.

He stayed in the ICU overnight, came off the blood pressure support the next morning, and was moved back up to the inpatient wing the next day, stable.

We had a lot of visitors that day and I can’t thank everyone enough for showing up. In the most terrifying moments like that minutes turn into days, all you can think about is the worst case scenario, and having people there to talk with really helps time keep moving.

This was the day the whole plan had been waiting on. Isaac had a cardiac catheterization, where they go in through a vein and take real measurements and pictures from inside his heart. It’s the information the surgeons need before anyone decides what comes next.

He came through it with no complications.

I couldn’t be there in person sadly. I caught the same cold Isaac had, and the last thing I’d want to do is infect another sick kid in the cardiac unit so Mom took over at the hospital. We did a conference call with the cath docs once it was over.

The results came back with some good and some that needs fixing. The main measure of his heart doing its job came back normal (a good solid squeeze), and so did the pressures in his lungs, which matters a lot for keeping his options open down the road. The things that need fixing are mechanical: a narrowed spot in the path out of his heart, a couple of small leaks in a patch from his first surgery, and the leaky tricuspid valve we already knew about.

Now his case goes in front of the combined heart and surgery board, where the cardiologists and surgeons sit down together with all of it and decide the path forward. A few directions are on the table. Surgery to repair the valve and the mechanical issues, moving him from the IV medicine onto pills he could take at home, and they’ve mentioned a heart transplant as one possibility as well.

The plan was to give the new meds 4-5 days to work and then have a heart cath to check the pressures and get better images of his heart. If all looked well we would switch from IV only meds to oral medication and go home to work out a plan forward and see if surgery was still on the table.

Well day 5 Isaac spiked a fever and looked and felt very sick. Isaac has a special IV line called a PICC that runs up near his heart, and those lines can get infected, which is dangerous for a kid like him. So the team didn’t wait around. They drew blood cultures from two different spots to find out whether the line was the source, and started antibiotics to cover him while the cultures grew. Those couple of days were hard, it seemed to us and the docs that infection was the most likely cause giving timing and the sudden onset.

It turned out to be a simple virus though. The blood cultures stayed clean, the line wasn’t infected, and they stopped the antibiotics.

This pushed the cath procedure back another week though.

Isaac is responding well to the meds. They’ve pulled around three liters of fluid off of him over these few days, which is a lot for such a little body. His face isn’t puffy anymore, his belly is softer, and his liver has come way back up toward where it belongs. He told us himself that he “feels much better.”

The one that got me was his lips. They came in dull and would turn blue easily, and now they’re a vibrant, healthy red. A repeat echo showed his right ventricle’s pumping function had improved significantly.

This one had been building for a few months. We’d been emailing Isaac’s cardiology and electrophysiology teams more than a few times about changes we were seeing. His energy was dropping. He was napping more. He was having headaches and tummy aches that kept sending him to the school nurse. At first we wondered if it was his medication, since his doses had changed not long before, and the teams looked into it with us. We took him to the pediatrician who xray’ed him and gave him medicine for heartburn and a laxative.

During this time his care team presented him to the Surgery board and we were scheduled for a major open heart surgery for end of June. They wanted to replace or repair his Tricuspid valve, replace the pulmonary valve that would have needed replaced in a few years anyway and do a maze procedure to help stop the flutter episodes.

Then over about a month, we noticed his belly looking bigger and his energy dropping further. He wasn’t swelling anywhere else and his color and breathing seemed alright, so we kept watching it. At a checkup his pediatrician could feel his liver seemed lower, and that got us in to see his cardiologist.

After an exam and an echo, the picture came together. His liver was down about 5 cm below where it should sit, and the chamber of his heart that does the hard work, his right ventricle, is failing. That was causing fluid to back up into his belly and his liver.

They admitted us to the cardiac ICU. He was started on milrinone again, along with IV diuretics to pull the extra fluid off of him.

Two months later, the flutter was back. This time it was happening for a month and some setting on the pacemaker caused it not to notice. Looking back he did sleep extra long a few days during this period but nothing that caused concern, was a bit more fussy.

First they did a Transesophageal Echocardiogram, an ultrasound, to make sure no clots had formed. That’s a big risk with flutter: when the top chambers race instead of squeezing, blood can sit still and clot, and you don’t want to reset a rhythm with a clot in there. The echo showed his heart was clear.

Then, instead of the shock paddles, they used his own pacemaker. They programmed it to briefly pace his heart faster than the flutter itself, which breaks the racing loop and lets the normal rhythm take back over. It worked.

His sotalol went up again, and they added a new blood thinner for three months as a precaution. We were home the night of Christmas Eve, which felt like its own gift.

Three rhythm admissions in one year tells you something, and we knew it. The team had started talking about the bigger picture, his leaky valve and whether it was time for another surgery to address what’s driving these episodes. Those conversations would carry into the new year. But that Christmas we had him home, in his own bed, rhythm steady.

Seven months later, the flutter broke through again, and we were back for another cardioversion.

Same routine as March. Sedation, the timed shock, rhythm reset, our boy back to normal before he even knew anything happened.

The plan coming out of this one was to increase his sotalol. That’s the chess match with rhythm problems: the medicine holds the line for a while, the heart finds a way through, and they adjust.

You might remember the very first sign anything was different about him came before he was born, when a checkup found his heart racing over 200 beats a minute. Fetal atrial flutter. It settled before birth with meds and stayed quiet for years. But his pacemaker logs everything his heart does, and a couple years back his checkups started catching episodes of that same fast rhythm. He was put on a rhythm medicine and we watched it.

This week his heart went into flutter, the top chambers of the heart race in a fast loop and the heart can’t fill and pump right. We didn’t see any signs of this but his pacemaker sent an alert to the electrophysiology team. They admitted him for it. The fix is called a cardioversion: they sedate him, deliver a carefully timed shock, and it resets the rhythm. It worked on the first try.

He came home on a stronger rhythm medicine called sotalol to keep the flutter away.

About a year after Isaac’s big surgery, we were back at the hospital, this time for a much smaller fix, and I want to write it up because it shows the other side of congenital heart care, the kind where they repair something without ever opening his chest.

A little background first. During his double switch, part of what the surgeon built was a set of tunnels inside his heart called baffles, which reroute the blood to where it needs to go. At a checkup, they found that one of those tunnels, the one carrying blue blood back from the upper body, had developed a small leak, about 7 millimeters. The leak was letting some of the blue blood cross over and mix in with the red blood heading out to his body. That kind of leak can pull a kid’s oxygen levels down, and left alone it can grow, so the team wanted it closed.

The fix did not mean another open heart surgery. They did it in the cath lab. They go in through a vein in the leg, thread a thin tube all the way up into the heart, and place a small device called an occluder right in the leak, like a tiny plug that the heart tissue then grows over and makes part of itself. Isaac was under anesthesia for it.

It worked. The device seated right where it needed to be, the leak was closed with no leftover leaking, and it sat clean without pressing on anything around it. Just a one night stay and we were back home.

On Day 26, March 20th, we finally took him home. Not unplugged from everything, mind you. He came home with a portable wound vac over his incision to help it heal, and a list of medications longer than my arm: heart medicine, a diuretic, a seizure medication as a precaution, methadone he was still being weaned off of, and a few others. Going home from a stay like this doesn’t mean it’s over. It means the hospital part is over.

It also doesn’t mean your kid comes home the way they left. Before the surgery, Isaac had been walking since he was ten months old, all over the house, into everything. A month flat on his back in a hospital bed took that from him. He came home unable to walk, and we had to reteach him, the same little milestones all over again, holding our hands, cruising the furniture, first steps for the second time. Nobody really warned us about that part. If your child is going in for a long stay, know that the recovery doesn’t end at the hospital doors. The strength and the skills come back, his did, but you’ll be earning some of them back together at home.

After everything, the marathon surgery, the open chest, the kidney scare, the seizure and the stroke that turned out not to be one, the infection, the clots, a pacemaker, and learning to walk twice, we walked out those doors with our boy. Thinner, scarred, and with some hardware on board, but alive, and with us. They told us at the start to plan for seven to ten days. It took 26. If your family is headed into one of these surgeries, hold your plans loosely and hold your hope tight.

Two days after we got home, he was back at the clinic just long enough to have his sutures and the wound vac removed. The incision had healed clean.

Note: This was all during peak covid. Visitors were not allowed and the last thing we wanted to do was have him fight off covid while being so sick already. We had some family visit outside once or twice but it was largely just us.

On Day 16 Isaac hit another milestone: they weaned him off the IV heart medicine called milrinone that had been supporting his heart since surgery, and moved him onto a heart medication he could take by mouth. His blood pressure held steady, which was a big checkbox on the road home. Getting off the IV support and onto oral meds is one of the main things that has to happen before they’ll let you leave.

Somewhere in this stretch is the story I most want every heart parent to read, because it’s the most important lesson we took from this whole stay.

Isaac had developed clots, one in a vein up near his collarbone and one in his leg. For a few days the teams went back and forth about whether to treat them with an aggressive blood thinner. The problem was the small bleed on his brain. A blood thinner helps the clots but can make a brain bleed worse, so it was a real debate, and they kept doing CT scans to keep checking on the bleed before making the call.

Then one night a nurse came in and said they were adding a new medication. I asked what it was. It was the aggressive blood thinner. I said I don’t think that’s correct, because on the last CT they still saw a bit of blood on his brain. There was some back and forth, but I insisted they check with the attending before giving it. They checked. The attending confirmed I was right. The chart was wrong, and they did not administer it.

I’m not telling these stories to knock anyone. The people who took care of Isaac saved his life, and we’re grateful beyond words. But a hospital is a lot of teams and a lot of departments, and they’re all human. Things move fast, charts get updated by many hands, and the one constant in your child’s room, the one person who is there for all of it, is you. You are your child’s care manager. Ask what every medication is. Ask why. If something doesn’t match what you were told, say so, and don’t be afraid to ask them to double check with the attending. And when they’re handling your kid, you’re allowed to say slow down, be careful, he’s connected to things. Those questions are part of keeping your kid safe, and nobody knows the whole story of your child’s stay like you do.

Even the home stretch had one more wobble in it. About a week before discharge, the monitors caught eight beats of a fast rhythm from the bottom chambers of his heart. They interrogated his pacemaker and did another echo, and both came back reassuring. After the month we’d had, we were almost getting used to the pattern: a scare, a workup, and an answer we could live with.

This is where I stopped the daily logs. The first nine days were such a whirlwind. After Isaac turned the corner, the days started to look more alike, in a good way and we focused on getting Isaac better. So I’ll cover the rest of the stay in a few posts, including a couple of stories I think every parent going through something like this needs to hear.

Delirium:

After the ventilator came out, it was a stretch of slow recovery. The tubes started coming out one by one, the chest tube, the drains from his stomach. He was weaned down off the heavy medications a little at a time. His voice came back, quiet at first, like a newborn cry, nothing like the old cry that could wake the dead, but it came back. The respiratory infection he’d picked up from the breathing tube turned out to be MRSA in his airway, so that needed treatment and isolation precautions, one more thing on the pile, but they got ahead of it.

There’s one part of coming off sedation that nobody prepared us for, and I want other parents to know about it, because there’s something you can actually do to help. After being sedated for so long and on so many medications, Isaac developed delirium as he started coming out from under it all. He would scream, his eyes looked spaced out, and he just seemed terrified all the time. It’s an awful thing to watch, your child awake but not really there, scared of everything and recognizing nothing.

What really helped was the familiar things. We surrounded his bed with his stuffed animals from home, even before he could grab them, even before we could tell he recognized them. We read to him by the bed before he could talk or move much. And there was a picture frame collage at the house that he would always point to and smile or laugh at. When we brought that picture in and showed it to him, it was like a trigger that helped pull him out. He smiled and enjoyed it, our boy surfacing again. He was in and out of it for a few days, but introducing those familiar pieces of home really helped bring him back to us. If your child is facing a long sedation, bring the familiar things early, even when it seems like they can’t take them in yet. Part of them can.

Huge Scare, kicking out his pacer wires

In the early hours of one morning, a day or two before his pacemaker surgery, they came in to get a chest x ray. To do that, they lift him up and slide a board underneath him. He was still connected to the external pacemaker, the machine outside his body that was keeping his heart beating through temporary wires, and he was upset and crying and kicking the way any baby would. They weren’t careful and patient enough with him, and he kicked one of the pacemaker wires and pulled it out of the device. He went from screaming to instantly laid flat and out. His heart had no rhythm of its own to fall back on. They reacted fast, plugged the wire back in, and he came right back and was fine. But I will never forget watching my son switch off like that.

His heart block never went away. We’d been holding out hope after the electrophysiologist saw signs it might be improving, but past a certain point it usually doesn’t resolve, and his didn’t. So on Day 14 they placed his pacemaker generator. In little kids it goes in the belly area rather than the chest.

A quick recap of the last few days, with a more detailed timeline to come later.

Tuesday (day 8) was his birthday, and we’d hoped to get the ventilator out, but couldn’t, because he seemed to be developing a bit of a respiratory infection, likely from the breathing tube, and was gathering some fluid. They did an ultrasound to check his lungs in case they had to drain fluid, and there wasn’t much there. But they found a large collection of fluid in his stomach pushing up on his diaphragm so he couldn’t take deep breaths. He already had one tube draining his stomach from right after surgery, but it wasn’t reaching all of it, so they put in another, and it drained a lot.

His platelets were low, which is common after something like this, so they gave him a transfusion of plasma and platelets to help his blood clot properly. After some of that fluid came off, they were able to dial his oxygen support down, and he was breathing better. Today they got the ventilator out, which is amazing. He looks so much smaller now that all that water is coming off. They also took him off fentanyl and moved him to methadone to manage coming off it safely, since you can’t stop it all at once after a week.

When the tube came out, he quietly cried, and even though you can’t really hear it yet, it’s there. Over the next day or so he’s set to lose his chest tube and the tubes to his stomach. So we’re making a lot of progress.

One more big thing. The plan had been to put in a pacemaker on Friday if he didn’t come out of heart block, since after about seven days it usually doesn’t go away on its own. But an electrophysiologist tested some things and found Isaac’s heart block seems to be getting better, not worse, which is not common after that point. There’s a small chance, but a chance, that he won’t even need a pacemaker, which would be amazing. So they decided to wait and give him more time.

We talked to neurology, and because they had the follow up CT scans, they now think it may not have been a full stroke after all. It could have been a shadow from the EEG leads on his head that made it look like one. On the two follow up scans, they didn’t see it at all. They can’t fully confirm it for a few months, because he can’t have an MRI yet due to his pacing wires, but that’s fantastic to hear. He does have a very small bleed in the back of his head, about the size of a fingernail, which is likely what caused the seizure, but that should reabsorb on its own like a bruise.

Other things are looking good too. His blood pressure is staying stable. Things are looking much better this week. Hopefully in the next day or two they can get his breathing tube out and we’ll finally hear his little voice. With a breathing tube in, he can look like he’s crying or coughing but you can’t hear anything, since it compresses the vocal cords. His platelet level is still a little low, so we want that to come up.

We got another echo to see how well his heart is pumping. Much more positive day. Sounds like there may not be any brain damage. It’s been a roller coaster, and the doctors are finally seeming really positive. We’re not out of the woods yet, he’s still critical and on a lot of medication and sedated, but thankfully with everything he’s on, he won’t remember any of this. I’ll let you know what the echo says. I just wanted to get some good news out there. Tomorrow he turns 1 <3.

We hadn’t put out an update in a bit, because we had some big setbacks this past week. It made for a very tough stretch. We never went into this to scare anyone going in for this kind of procedure. We just wanted people to have an idea of what it can look like. What we prepared for was not what happened. But today, we’re finally seeing some light after a very dark week. So I’ll walk through it the way it happened, from the journal I’ve kept.

What we were told to expect was a procedure with maybe a five percent risk of death, with most kids going in not as healthy as Isaac, who had gained weight the whole time and stayed mostly asymptomatic. We came in Tuesday the 24th. They took him back in the morning, first incision around 9:30am. We were told to expect about a three hour procedure, five hours total before we saw him.

Instead, we didn’t hear back until 4:30 that afternoon. By the time it was done, we were about eleven hours in. We saw him around at 10pm that night, chest still open under a patch. It was one of the most frightening things you’ll ever see. His blood pressure was low, and they were giving him medicine to bring it up.

That brought us to Wednesday. They lowered his sedation around 7 in the morning to look for movement, and it took a while, but he moved. There was a real fear of brain damage due to extended time on bypass. His blood pressure was still low, and he was very swollen all over, his liver, his face, everything, with a fever they were treating with a cooling blanket. They couldn’t close his chest yet because of the swelling and the low pressure.

Thursday, his creatinine, a marker of kidney function, was high, and he wasn’t urinating much. The surgeon closed his chest on Day 3 and put in permanent pacing wires, since he was still in some heart block. They put a tube in his stomach to drain fluid since he was retaining so much. We were told he’s not where they want him to be, but they’ll get him there, even if it takes a few days.

Friday, his kidney numbers bounced up and down all day. They told us he had a lot of what they call third spacing, holding water in his tissues instead of his veins, which was keeping his blood pressure low. By the afternoon his kidney function looked much better, he was urinating more, and his oxygen improved. They cut back his sedation, and at 5:30 that evening, for the first time since Tuesday (day 1), we saw him move. He moved his legs and his mouth. That filled us with a lot of hope.

Then on day 5 at 4am, they woke me (dad) to say Isaac had a seizure in the night. The EEG pointed to his left frontal area, which can indicate a stroke. They did a CT scan, and a little later neurology told us he’d had a small to moderate stroke in that area, likely while he was on bypass, which is a known risk. There was also a small amount of blood pooling in the back of his head. As you can imagine, that was a very scary, very tough day. They explained plasticity to us, how children’s brains are still developing and can often work around damage. They couldn’t tell us much yet because he was still on so much medication and not awake yet.

But that afternoon a second CT showed no extra blood and no worsening of the stroke area. And at 7 that evening, his anti seizure medicine wore off and he opened both eyes, kicked both legs, and moved both arms. That gave us so much hope, because stroke is one of the main causes of fatality in these surgeries.

Then around 9, one of the doctors came in and worked with his pacemaker, running some tests with different settings. He found that Isaac’s underlying rhythm wanted to race, and the pacemaker was fighting against it. He was able to get it out of that arrhythmia so the two stopped competing and started working together. He brought his heart rate down, his blood pressure held steady through the night, and they were able to cut back some of the medicines. Honestly, without that doctor catching it, I’m not sure he’d have turned the corner the way he did.

This morning he looked much better. Less facial swelling, great kidney numbers, urinating well, oxygen better. They brought him down a little more on the pain medicine, he opened his eyes more, and he seemed to calm to our voices. They put a wet sponge to his lips and he sucked on it, and when they took it away he opened his mouth wide because he wanted more, and reached his hand up. That one moment filled us with so much joy and gave us something to cling to.

If you’re going through something like this, try to hold onto hope. Mom and I fell into despair, just sobbing in the corner of the tiny ICU room until this moment when he finally opened his eyes and drank from a sponge. I can count on one hand how many times I’ve cried in the last ten years, but this week I could fill buckets. In a few day’s it’s Isaac’s 1st birthday, and with any luck the tubes come out and we get to see our boy actually awake.

The good news is the way they corrected it is good. The right chambers are now pumping to the right sides of the body. The whole reason we did this was the risk of that ventricle giving out, and that part is corrected. So now it’s about recovery: healing from the open heart surgery, getting closed up, and making sure his heart accepts the conduit they placed.

For the long term, because he had to have a conduit placed, that conduit doesn’t last forever, usually around ten years, and since he’s little and growing he’ll probably have to go in again to swap it out when he’s older. But they told us this is the biggest surgery he’ll ever have to have. The future ones will be less invasive.

They put permanent pacing wires in. If his heart comes out of heart block and can keep a good rhythm on its own, they won’t need to use them, and they’ll just leave them tucked under his skin so they don’t have to go back into his chest later. If it doesn’t, he’ll get a pacemaker, which in a small child usually goes in the belly area. They told us you can live a long, good life on a pacemaker.

One thing I learned that I’ll pass along: with this condition, it’s really all about the associated anomalies. If you only have ccTGA with nothing else, you might never need surgery. It’s the things that come with it, like the pulmonary stenosis and the outflow obstruction, that cause the bigger issues over time. We appreciate all the prayers.

Yesterday our son went in for the double switch to correct his ccTGA and its associated anomalies.

The surgeon went in hoping to do the Rastelli, but when he got there, the VSD, the hole in the heart, wasn’t like one he’d ever seen. It didn’t lead out where it normally does, so the Rastelli wasn’t an option, and he pivoted to the Nikaidoh procedure, which was always one of the possibilities. That part involves putting in a conduit to replace the pulmonary valve, since Isaac had pulmonary stenosis. That went well. He was able to switch the two bottom chambers and connect everything where it needed to go.

Then they started taking him off the bypass machine, and he went into heart block, even with the pacing wires connected. Heart block isn’t uncommon for kids with ccTGA, their electrical system is a little different. They put him back on bypass for a while and tried again, and he was still in complete heart block. So they had to find out what was causing it. They put a catheter in and did imaging, and along with an ultrasound they found that one of the coronary arteries, the one the surgeon hadn’t touched, was kinked in a way that wasn’t allowing blood flow. So the surgeon went back in, moved it, and reattached it.

It was a long day. The first incision was around 9:30am, and what was supposed to be about a three hour procedure ended up taking nearly twelve hours. That long on bypass causes swelling, so they left his chest open to drain and to let the swelling go down before closing him up, which they expected to do in a day or two. We got to see him around 8:30pm that night. It was a shock. They prepare you for the tubes, but seeing him with his chest open under a patch, swollen like a balloon, was hard.

Today was a roller coaster of keeping his blood pressure where it needed to be, his rhythm steady with the pacemaker, and his temperature down. After big surgeries like this, their temperature runs high the first couple of days, so they had him on a cooling blanket with the room kept cold. This morning they lowered his sedation just a little to see him move and twitch, to check his brain function. It took a little longer than they liked, but it happened, which was a relief, and then they put the sedation back up.

His blood pressure is looking better tonight. They’re hoping to close him up tomorrow and start bringing him off sedation so he can wake up a bit and get onto the good path of recovery, as long as that blood pressure holds.

We wanted to record this because online we didn’t see much about ccTGA. We found one or two stories here and there, and reading them really helped us, since it’s such a rare condition. So we wanted to share our son’s situation and document how it goes. If you’re going through it, you’ll have at least a small idea of what someone else went through. Every case is very different, and there are a lot of different avenues for care, but this gives you an idea of what our situation was like.

A little of his story first. This all started around 32 weeks, when Mom was pregnant. We went in for a normal checkup, and they found the baby’s heart rate was up around 200 beats. They sent us for an ultrasound, it didn’t look quite right, and we ended up at the hospital for about five days with a lot of ultrasounds and echoes. They called it fetal atrial flutter, his top chamber beating very fast. They gave Mom medicine, and thankfully it brought his heart rate down. That’s also when we found out he had ccTGA and a VSD. He was born full term at 39 weeks, a big baby at 9 pounds 4 ounces, which is great, because a lot of heart babies come early or small.

He spent his first five days under observation, and we got sent home on no medicine at all. They gave us a stethoscope and told us to listen to his heart each day and watch for him to turn blue. The first weeks were the hardest because we had no idea what to expect. It felt like sitting on a time bomb. But nothing happened. He gained weight just fine. He ate constantly, walked around everywhere, got tons of teeth, started walking around ten months, and his favorite thing in the world was Baby Shark.

We later learned he also had an Ebstein’s anomaly of his tricuspid valve and a left ventricle outflow obstruction. Early on they were thinking about a surgery called a PA banding to strengthen that ventricle, but it turned out he had just the right amount of natural obstruction, so he self banded and didn’t need it. They just watched it over time.

So now we’re here for the big one, the double switch. Best case, he has the surgery and we go home in about a week. They told us to plan for seven to ten days. He may need a pacemaker. They say about twenty percent of kids with this need one at some point because their electrical flow is different, and we’d already seen a little of that. We’ll keep everyone updated. If you have a child going through this, reach out and let us know.