ToF/PA Jessica's heart defects: description of them and explanation of her surgeries

I received this email on one of the heart support groups I belong to. ToF/PA is: Tetrology of Fallot and Pulmonary Atresia. I thought I would post my answer to this email here since it explains what Jessica's heart defects are and what surgically has been done to help her. I also put down some of the major complications that occured during or after each surgery. I spent quite a bit of time writting it and I appologize for any spelling or grammar mistakes... it is 2:45am afterall! It's a bit of a long read but well worth it - every time I explain to someone everything that Jess has wrong and all the surgeries she's had - and especially the complications she's gone through and how she has RECOVERED from them... it helps rekindle my faith in God that He loves her and He is granting her life - the doctors sure don't have any other explanation that she's still alive! ;-)

The question was:
---Anyone here have child with TOF/PA and the pulmonary artery AND the
pulmonary valve are absent?
What did they do for the child and WHO did it?

Karin---

Ahem....
Jessica! How could you forget, Karin? lol!

The Pulmonary Artery is supposed to be shaped basically like a T. The top piece of the artery goes to each lung and then the lower part reaches down to the right ventricle. Jessica is missing the part that reaches down to the right ventricle of the heart - therefore there is absolutely no direct connection between the heart and lungs - therefore no valve either. Jessica has a huge VSD so the blood that would normally go up the pulmonary artery to the lungs goes through the VSD and mixes with the blood in the left ventricle and up through the Aorta. Part of the ToF is an over-riding Aorta meaning it sits right on top of the VSD. Sometimes there is also an ASD present but we never found one with Jess. Anyway, there are usually a lot of collaterals or MAPCAS (not sure exactly what that stands for) that come off the Aorta and go directly to the lungs. The blood that gets to the lungs is mixed (oxygenated and unoxygenated) as well as the blood that gets out to the body. That's why they are cyanotic. Another thing that has been a major problem with Jessica is that the piece of PA that reaches from lung to lung is extremely narrow and stiff - more like a vein than an artery. Also, Jessica's piece of pulmonary artery branches out to only 1/3 of each of her lungs. The collaterals from the aorta provide the rest of the 2/3 of each lung.

Here is what they have done for her (or TO her - depending on my mood)
First heart surgery (when she was 5 months old - they waited because she was so sickly and weak they wanted her to gain some weight in order to survive surgery):
A central shunt was placed between her Right Ventricle and Aorta. This gets blood flowing through that piece of pulmonary artery where it had none. This helps get more blood to the lungs and the hope if that if blood is flowing through it, it will grow (if it is too small - like Jessica's). Complications: blood clot went to speech area of brain causing right-sided weakness and much difficulty learning to speak... she learned sign language when she was 18 months old and later learned to talk. She regained strength on her right side with therapy too.

Second heart surgery (when she was 3 years old):
They replaced the shunt. The feeling was that she had out-grown the first shunt and her piece of PA that goes from lung to lung was still too small to handle complete repair). Complications: The original central shunt had fused into her sternum and it was cut when they opened midline. She hemorrhaged from the site so no blood was getting to the lungs at all. She went between 7 - 8 minutes without getting oxygen to her brain while they finished opening and placed her on bypass. During that time all the scar tissue in the area bled too and she hemorrhaged for several days - nobody expected her to survive. Later when she was finally extubated, she was blind, had gaze paralysis (her eyes were stuck towards the right) and her left side (formally her stronger side) was paralyzed. Fortunately as the swelling went down in her brain she regained most of her eyesight and with therapy she re-learned how to roll over, sit, talk, crawl and walk. She also developed a blood infection that had to be treated with IV antibiotics. Very, very difficult times.

When Jess was almost 5 years old we were told that if she were to ever have complete repair we needed to consider some new alternatives. Since Jessica's pulmonary artery didn't branch out to all of the lungs if they did complete repair, she would only have the use of 1/3 of each lung. So here's how things followed:

Third heart surgery (when she was 5 years old - and I had a 2 month old baby):
Unifocalization of the collaterals on the right side. At the time this was a new thing. Remember that Jess has collaterals coming from the Aorta and go directly to the lungs. What they did was opened Jess on the right side under her arm and take the collaterals that were connected to her right lung - disconnect them from the aorta, sew them together and attach them to her piece of pulmonary artery (that goes from lung to lung). Now the collaterals go from the piece of pulmonary artery to the lungs, providing her with pulmonary artery branches of sorts. Complications: nemothorax - air bubble pressing on her lung causing it to collapse. After awhile it resolved itself but was very scary.

Fourth heart surgery (7 months later just after Jess turned 6 yrs old):
Unifocalization of the collaterals on the left side and placement of a conduit between her right ventricle and piece of pulmonary artery. The PCs and the surgeon really debated as to whether to put a valve in the conduit or not. The cath suggested that the existing piece of pulmonary artery would not handle a valve. There had also been a case similar to Jessica's where they had placed a valve in the patient and he crashed post-surgery. This had been in another state and they told me that the boy was rushed back into surgery and they had to do emergency repair. Fortunately his pulmonary arteries were large enough to handle all the bloodflow and he was ok. They KNEW that Jessica's would not handle all the bloodflow just yet so they finally decided not to put in a valve.

This was the surgery when the came out and told us that her pulmonary artery (the piece that goes from lung to lung) had not grown and that she would never receive complete repair. They went on to mention that most kids who live off of shunts like Jessica don't usually live past their mid-teens. They either get an infection they are too weak to fight off or they get too "blue" to survive. (extremely difficult news to digest)

Fifth heart surgery (1 year later - and I was 6 months pregnant! ugh):
The conduit that was placed between the RV and PA was acting as a vacuum and causing the blood to accumulate into the RV of the heart. Jess was dying. The PC and surgeon argued in front of Karl and I about whether to replace the conduit with one with a valve or to take down the conduit or whether to even do surgery or not. After much discussion and reviewing her cath, the surgeon called us at home and said that he felt he should take it down or not even do surgery at all since she had suffered strokes and many other complications. He left it up to Karl and I to decide whether to even do surgery or not. We decided to take down the conduit to help increase her quality of life and hopefully give her more time. Complications: As they were putting in the line in her neck, the artery tore - just like a run in a stocking is what they told us. They had to stitch it up and place another line in the artery in her left side. We worried for a couple of days as to whether she had an aneurysm there or even a bloodclot inside the artery waiting to break off and go to her brain (remember, we've been through that before). Fortunately neither was the case! Also, after surgery Jess was vomiting and they had to keep her intubated an extra day in case she inhaled anything... they could easily suction out her lungs if she was intubated. I was hormonal and not a happy camper. Fortunately she recovered very quickly once she was extubated.

So.... in conclusion, Miss Jess STILL had ToF and PA. We were told that if Jess had been born just 5 years earlier she would have been deemed inoperable and she would have died before her first birthday (according to her CT surgeon and PC). Amazing, isn't it?

We have discussed at length the possibility of a heart/lung transplant. The surgeon doesn't feel that she would make it off the operating table with her history of bleeding and she would have lesions everywhere. Karl and I have never felt comfortable with a heart/lung transplant but we discussed it anyway. Don't get me wrong, we are not against transplants, we just have not felt that Jessica would survive such a surgery and we don't want to be the ones deciding when Jessica dies - God can have that job - and He has blessed us so much more time than we ever expected.

Sorry this was so long, but you asked for it! lol. Now Karin, if you are passing on this information to new heart parents, please take out all the complications, ok?! No need scaring someone 1/2 to death! lol Also remember that the techniques have greatly improved since Jess was born EIGHTEEN years ago!

Here is a site that has a picture of pulmonary atresia. If you run your curser over the picture, it shows you what a normal heart looks like. With Jessica the bottom part of the T shapped pulmonary artery does not even exist.
She also has Tetrology of Fallot Her combination of defects is rare enough that I have not found any websites that have them together. If anyone knows of one, PLEASE let me know about it, ok? I would love to see it!