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Pulmonary Atresia with VSD

Jessica has pulmonary atresia with vsd. She has other problems as well. Jessica has very narrow pulmonary arteries that go from lung to lung. Also, the pulmonary artery branches only connect to about 1/3 of each lung. The rest of each lung gets blood from collaterals that come off the aorta.

I took a couple of heart and lung diagrams and made the appropriate changes to illustrate what Jessica's hear and pulmonary artery defects are compared to what's normal. I also included what was done during each surgery. The following diagrams are NOT profesional ones but they are as accurate as I can make them. The pulmonary artery branches and collaterals are not in the exact positions that they should be, but just give a better understanding of how Jessica's are so different than they should be.

Normal Heart with Pulmonary Arteries connected to the Lungs

LA=left atrium; LV=left ventricle; RV=right ventricle; RA=right atrium

Jessica's Heart and Lungs at Birth

If you'll notice, Jessica's heart has a hole between the two ventricles. This is called a ventricular septal defect (VSD). She has an overriding aorta where the aorta instead of sitting right over the right ventricle, is right over the VSD. She has no pulmonary valve or the piece of pulmonary artery that connects to the heart at all (pulmonary atresia). The piece of pulmonary artery that is black, goes from lung to lung and never even had blood going through it until after the first surgery. It also is only connected to about 1/3 of each lung and very, very narrow, not big and wide like pulmonary arteries should be. The purple vessels that branch out to the lungs are collaterals and were connected to the Aorta, which is the artery that takes blood to the body.

Jessica's Heart and Lungs after 1st Surgery

During the first heart surgery, a shunt was placed between the Aorta and the pulmonary artery to get bloodflow through that artery and try to force it to grow. Jessica had a blood clot break off and go to her brain during surgery. This caused her first stroke.

Jessica's Heart and Lungs after 2nd Surgery

Jessica had out-grown the shunt from the first surgery and needed a bigger one put in. When the sternim was opened to do the surgery, the first sunt was accidentally cut and she hemmoraged out. She also had a lot of extra scar tissue that bled. This caused her 2nd stroke. The first shunt was removed and a second, larger one was placed where it would be out of the way for upcoming surgeries.

Jessica's Heart and Lungs after 3rd Surgery

Since Jessica's pulmonary arteries didn't branch out to the lungs very well and it isn't possible to attach shunts to lungs, the doctors decided to use the collaterals that she had in a new (at the time) procedure called "Unifocalization of the Collaterals". They disconnected the collaterals from the Aorta and connected then to the pulmonary artery, trying to use them as pulmonary artery branches. They only did the right side, because in order to do it, they had to enter under the right ribs. Jessica suffered a collapsed lung, but shortly recovered and didn't suffer another stroke, thank heavens!

Jessica's Heart and Lungs after 4th Surgery

Seven months after the 3rd surgery, Jessica was back to have the "Unifocalization of the Collaterals" on the left side and also have a conduit placed between the Aorta and the right ventricle. This would be the first time that her heart would be directly connected to the pulmonary arteries. We were hoping that Jessica's pulmonary arteries had been growing and that she could accept the conduit and possibly have complete repair within the next year. Unfortunately, the surgeon came out very discouraged and told us that her pulmonary arteries had not grown and that she could never be repaired.

Jessica's Heart and Lungs after 5th Surgery

Over the next several months Jessica began to deteriorate very quickly. She was getting so "blue" and using a lot of oxygen when she hadn't used the oxygen much before. Finally a catheterization was done and to eveyone's horror, the conduit was acting more like a vacuum and the blood in her pulmonary arteries were being suctioned back into her right ventricle and she was in great distress. If left alone, she would die soon.

So it was left up to Karl and I as the parents to decide whether to risk doing another surgery and the complications that we had become all too familiar with or choose compassionate care. We also discussed heart/lung transplant extensively. Jessica would not be a good candidate because of her bleeding problems and her previous strokes. That too, was left up to us as parents whether to even put Jessica through all the testing and we knew that heart/lung tranplants are very difficult and we didn't know what Jessica's quality of life would be like. After much prayer we decided to have the surgery to disconnect the conduit. The surgeon agreed not to make a large incission mid-line, she had already been opened there 3 times. The surgeon made a small opening in between her ribs and closed off the conduit. Because of all the years that Jessica's right ventricle has had to work so hard, it has become hypertrophied, (the wall has thickened) and the right ventricle is weak. We have felt very strong that she should not have a heart/lung transplant. Jessica has voiced her opinions about this as well. In fact she still says today that she is so glad that she will never have another heart surgery and NEVER have "that tube" (the ventilator - breathing tube) down her throat again. As you can see, all her blood mixes and in round-about ways some of the blood gets to her lungs. It is truly amazing that Jessica has the energy that she does. Most people with as little amount of oxygen in their blood that Jessica has, are usually bedridden, if even alive. Thank you for taking the time to look over all this information. It just proves to me again, how God has worked in our lives and we thank the surgeons and the pediatric cardiologist for being by our side for 13 years! They are truly amazing and loving people, too.

Copyright (c) 2001 Nancy Jensen