Z had a pretty rough first year of life. Always a small baby, we discovered when he was just shy of 3 months old that he had a heart defect preventing him from thriving. The story is long and involved, but since we're about to pick up where it left off, I thought I'd share the back story so that future updates would make sense.
Jan. 24, 2009: "Z has a Ventricular Septal Defect (VSD) in his heart. Essentially, there is a hole between the two sides that allows blood to pass unrestricted from one side to the other. That means that some of the oxygen-rich blood coming from the lungs is going to the side of the heart that sends it back to the lungs, rather than out to the rest of his body. So his heart is working overtime to compensate – both for the extra volume that is going to the lungs and for the decrease in oxygenated blood that the rest of the body needs. His lungs are affected by the extra volume and fluid is building up in them, so when he came down with a virus it hit him harder than it would have without the VSD. The virus also made his heart work even more, and in fact was a blessing in disguise because without it we may not have caught the VSD for another month or more.
VSDs are not common, exactly, but occur often enough that doctors are used to looking for them. Generally if the hole is small it can be detected just by listening to the heart, and will usually be caught in the first week or so of life. When it’s small, the blood sort of “squirts” through it, and that is audible on a stethoscope. Larger ones, on the other hand, offer less resistance to the passage of blood and therefore do not make as much noise. Z’s VSD is moderate to large, and was not caught in his standard checkups because it wasn’t audible. The virus that he caught caused his heart to work harder, as well as causing him respiratory distress. So when he started wheezing and worrying his mother enough that she took him to the doctor on a Sunday, the doctor who was listening to his lungs noticed that his heart was beating much too fast and sounded abnormal. He checked Z into the emergency department at the hospital and a cardiologist did an echocardiograph of the heart. Diagnosis: VSD. Further tests showed that it was moderate to large, and that the pressures in the chambers were not normal. In a healthy, normal heart, the pressure on one side is lower. In Z’s heart they were equal. This caused him to display none of the usual symptoms of congestive heart failure, which is what a VSD can cause when it goes untreated. So while he wasn’t in distress from the VSD, per se, if it had continued to go untreated it would have started to cause damage to the lungs from the too-high pressure on that side. In addition, the sheer amount of work the heart was doing was causing his body to burn more calories than he was taking in, which was causing him to become more lethargic, which was making it harder for him to eat, and a vicious cycle was born.
Z spent Sunday afternoon through Tuesday afternoon at the hospital, then came home for a few days. The calorie deficit, however, caught up with him despite our best efforts, and he ate less and less. Finally we determined that he needed to have more help than we could give him, and Thursday night we brought him back to the hospital where they put him on a feeding tube. Regular full feedings via the tube have helped build up some energy, and Saturday afternoon he was finally able to do one whole feeding on his own from a bottle. He’s still in the hospital, and the length of his stay this time around has not yet been determined.
Z is currently on some medicines that are helping to better regulate the pressures in his heart and pull some of the excess fluids from his lungs. He’s eating a high-calorie milk/formula mix to help try and get his weight gain back up to normal. Now, small VSDs frequently close on their own and don’t generally require surgery. But because the VSD is moderate to large, and because it is preventing Z from gaining weight, some type of corrective procedure will almost certainly have to be done. Worst case scenario is that he’ll have to have open-heart bypass surgery to close the hole. Best case scenario (other than the hole closing on its own, which is extremely unlikely) is that they will be able to thread a catheter through a small opening and into the heart via a vein to put a patch over the hole. Timeframes range from next week to several months from now depending on the procedure, though almost certainly before his first birthday. The deciding factors are going to be how much he’s able to recover and gain weight, and how well the medicines control the pressures in the heart."
Jan 27, 2009: "Lots of encouraging news...the doctors are confident that they can perform the minimally invasive procedure, which involves putting a catheter through a blood vessel to the heart. The catheter will deposit a small patch that will cover the hole and allow the heart to perform without assistance. The heart will eventually just grow over the patch, so there will be no need to remove it at a later date. The procedure is happening at a hospital in a nearby city. The cardiology department there works intimately with the one here, and we were transferred over this morning. Z is all settled into his new room and flirting with the new nurses already. Mike and I are staying in a Ronald McDonald House right down the road at night but will be in his room with him during the day. Surgery is tentatively (right now) scheduled for Thursday morning, so I'll send out another update when we find out how it goes!"
Jan 31, 2009: "Hooray!! The surgery went quite well, the doctor was very confident that the patch is securely in place. Z is in the pediatric intensive care unit (PICU) recovering right now. He's a sad sight, all hooked up to tubes and monitors and groggy from the meds, but hopefully a couple of monitors will be coming off in the next hour or so and we'll be able to hold him again. The plan is to keep him in the PICU overnight to keep an eye on his breathing since he's still recovering from his virus, but the nurses seem confident that everything will be just fine. If all goes well we may be able to bring him home tomorrow!"
Interlude: A two-week post-operative echo reveals that the patch has either shifted or was not fully covering the hole; another attempt will need to be made. He had been taken off the feeding tube at the hospital, but doesn't gain enough weight and is put on a feeding tube March 5th.
April 15, 2009: "Z had another surgery today to correct his heart defect. The original surgery in January helped, but didn't fix it all the way. They attempted to put another patch in on April 7th using the same method, but weren't able to get the right angle needed to place it properly. So today's surgery involved opening his chest and sternum to try and get a better angle. They were successful in placing a second patch, but doing so did not fully fix the problem. With the better view they had they were able to determine that he has at least 4 holes in his heart. January's surgery covered the hole that was visible at the time, but the high pressures just forced through other weak spots to create the additional holes. There was no way to cover all four holes with patches, and an attempt at stitching them closed was unsuccessful. That left them with only one option, which is putting a band around his pulmonary artery to correct the pressures in his heart. This is only a stop-gap measure to buy us time for Z to grow in a more normal fashion without risking damage to his lungs. He will definitely have to have another surgery in a year or two to remove the band (it will become too small) and hopefully at that time they will be able to fix the holes. In the meantime, though, he should be able to start catching up on his growth and be in most respects a normal child."
April 22, 2009: "We're back home, now...Z is on several medicines, and taking plain Tylenol around the clock, but he's laughing and smiling and playing with toys. His eating is getting better, so hopefully he'll start growing like a weed! Right now we're going into a waiting phase that'll last until the band on his artery becomes too small for him, at which point another surgery will be on the agenda. Until then we're just going to have all the regular pediatrician visits with the occasional check-in with the cardiologists. He can spend his time being "normal" for a change!"
May 23, 2009: Feeding tube is taken out (by Z!) and the decision is made to not put a new one in, pending how well he does on his own. He does fine, and no more tubes.
October 4, 2011: At a regular cardiologist checkup, the doctor is of the opinion that it may be safe to remove the band. The holes are smaller in relation to his heart (the holes are the same size as before, but the heart is bigger) and he feels confident that no other interventions will need to be done. Upon review with the other pediatric cardiologists, it is decided to go ahead with removal. Since removal requires opening the chest again in order to remove scar tissue, they plan to attempt putting in another patch while they have the opportunity, in a "we probably don't need to but we're in there already so let's do it to be on the safe side" frame of mind. Surgery is scheduled for Oct. 6th.