Heart Surgery Plan for Lily

We talked to the cardiologist today and I guess they did come up with a game plan when they met with the medical team. I guess they teleconference with many cardiologists from all over Canada and get input from this whole committee. Together as a team they have decided on which route will be the best for Lily.

The Dr informed us that they will do surgery next week. The day is yet to be determined, as they assess each day who is more critical than others. They do surgery 5 days a week, and sometimes on the weekend. Recovery period is approximately 2-4 weeks from date of surgery. Some of this recovery time may be able to occur in the Alberta Children’s Hospital in Calgary – we will wait to determine that later. They will then have to do another surgery at 4-6 months, and another at 2-4 years. So a total of three surgeries – assuming everything goes well. I will try and describe the surgeries, and the overall goal they are trying to achieve.

The overall plan is to re-route all the blood which would be coming from her body to the heart and re-route this deoxygenated (or blue blood) directly to her lungs instead of returning to the heart first. The blood then gets oxygenated in the lungs (referred to as red blood) and then would return to her heart via the normal path. This method of having the blood go directly to the lungs first, would essentially be letting the blood seep into the lungs vs. being pumped their by the heart. As you can imagine, blood flowing/seeping to the lungs vs being pumped into the lungs is less effective and not natural; especially for baby’s lungs. They have a tendency to resist the blood flow/seeping into the lungs, but as they get older they become less resistant (thus the bulk of the surgery needs to be done later). The goal described above is achieved normally in two surgeries, called the Glenn procedure (at 4-6 months) and then the Fontan procedure (at 2-4 years). In order to help get Lily’s lungs ready to accept the blue blood directly flowing/seeping to them, the doctors need to do a surgery sooner than later to prepare her lungs. They do this by restricting the blood flow to her lungs. Right now, she is pumping lots of blood to her lungs, but not pumping it out effectively, causing extra stress on her lungs. They need to essential rest her lungs or reduce the stress on them.

This is a picture of a TGA heart on the left (somewhat like Lily’s) and a normal heart on the right. Use them to understand which arteries do what. Aorta goes to the body, Pulmonary goes to the lungs:

TGA

Normal Heart

Typically with TGA, they would just switch the Aorta and the Pulmonary arteries. However, Lily’s right ventricle is too small and is not capable of pumping as it would need to. Thus an alternate procedure is required.

First step (done next week) is to take the Pulmonary valve and connect it to the Aorta. This will direct all the blood flowing out of her heart to the body (and none to the lungs). You may wonder, “how does she get blood to her lungs?” They will do this by putting a synthetic shunt (or tube) from her right ventricle and connect it to the pulmonary artery, which will pump it to the lungs, but a smaller amount of blood (just enough to keep her saturation (or blood/oxygen levels) where they are currently at. This surgery is called the Norwood or Sana operation. The shunt is only temporary and will be removed when she goes in for her surgery at 2-4 years.

At 4-6 months, they will do the Glenn procedure and take the SVC (superior vena cava, which is one of the main veins from the body) and connect it directly to the pulmonary artery; which will take blood directly the lungs.

At 2-4 years, they will do the Fontan procedure and take the IVC (inferior vena cava, the othe major vein from the body) and also connect it directly to the pulmonary artery; which will also take blood directly to the lungs. They will also remove the shunt they are placing in next week.

I know this is hard to completely understand without it being described in person with a sketch, so I hope you can follow. I would be more than happy to explain it all when we are talk in person and I can sketch it out.

If you have any questions, I will try and explain it or ask the questions. This surgery has only been done since the 80’s and thus they do not have anyone older than 30 who has had this procedure. Her quality of life will be good, as long as she does not feel like she disappoints people who might expect her to be an athlete. Brain or mental development was explained to us like this: She will likely be able to keep up with the grade level for her age. They did say that studies have shown that learning difficulties, attention difficulties, and behaviour problems are quite common with heart surgery children. I am not too worried about this as it will just take some extra parenting, patience, and extra love to help her achieve. I know the Lord has blessed her with special gifts to help her succeed and be happy. She will likely be on blood thinners her whole life, starting with Aspirin, and later after her last surgery something a little stronger.

Please continue to pray for our little Lily, especially as she will be having surgery soon. It is a little frightening for Leah and I, as it is easy for us to worry about possible negative outcomes as it is a fairly complex surgery.

For Now we will just continue to enjoy the Snuggles and precious moments we get to have with her.