Vascular Ring

Before discharge from Lurie we got some more news regarding James' heart. This was one area of his body we thought we had checked off the list as being fine, besides the VSD or ventricular septal defect which was something we may or may not have to deal with down the road.

The team had seen a narrowing of his esophagus during the initial swallow study. I had seen this too as one of the speech therapists pointed it out mid-study and the radiologist kind of brushed if off. I thought he really was brushing it off, but turns out they discussed it once I was out of the room. Their first thought ended up being correct, he had a vascular ring.

Fast forward a week or so after a CT scan of his chest when we were told the news. Our neonatologist and his fellow both came in to tell us and I had learned that whenever you're approached by more than one person the news isn't good. He told us they had found a right aortic arch with a vascular ring that would require surgical intervention likely sooner than later. In a nutshell, his aorta wasn't quite in the right spot and was going up and towards the back of his body and then downward instead of arching downward right away. It was also forming a 'ring' around his trachea (airway) and esophagus and as he grows will tighten -- this is why it will require surgical repair.

My initial response was anger, why were we just finding this out? He had had CT scans of his body before, he had had echos on his heart. There should have been multiple times were they found this and now, after nearly two months in the hospital and so so close to going home we were just being told. I was afraid this was going to hold us up from going home and honestly I was just so over being told bad news. The doctor apologized again and again and we had a good discussion about being kept in the loop more in the future, as I felt we hadn't been. Hard to come from a small hospital with about six babies at a time and being constantly communicated with in person step by step to a huge hospital with 40+ babies where, to quote the doctor, "your baby isn't the most acutely ill baby, we've got our hands full."

Again, our initial thoughts were that this was something that would have to be fixed right away. Our neo doctor I believe said within a couple months but that he was going to consult with the cardiologist to get more information. We then were told at around 18 months we could expect the surgery as this was when James would likely start eating solid foods like raw carrots which become an issue as the narrowing in his esophagus could block whatever he's eating and cause him to choke. Our thought is why wait, let's get this over with but they want him to be as big as possible to make the surgery 'easier' and safer. We were also told that this would not be open heart surgery and that they go in through the side of his chest and that the surgeon considers it a relatively routine procedure and that he's the top surgeon for this specialty. Great, but let's still do it sooner than later.

He also still has the VSD - ventricular septal defect- which is a separate issue altogether but due to the 22q, as is the vascular ring, and we were told would have to be a separate surgery to repair if needed. We see the cardiologist today and I'm sure will be updated on the timeline of things and the likelihood of surgery and repair for this defect.