Transposition of the Great Arteries with Pulmonary Artery Stenosis - need answer fast

In My Humble Opinion
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Family crisis. Looking for help finding studies, surgical approaches, etc to ask about in seeking a second opinion.

The mother, 27 year old Colombian in-law is 8.5 months pregnant. At random ultrasound on 1 Sep was referred to pediatric cardiologist with diagnosis of Transposition of the Great Arteries (TGA) with Pulmonary Artery Stenosis and Ventral Septum defect. Ductus arteriosus and foramen ovale are patent.

Explanation of TGA for the masses

[spoiler]Normal circulation returns blood to the right atrium which flows to the right ventricle where it is pumped to the lungs. Blood off loads carbon dioxide and pucks up oxygen int he lungs. Blood returns from the lungs to the left atrium which flows to the left ventricle and is pumped out to the body.

In TGA the plumbing is mixed up on the outlet end. Circulation returns to the body to the right atrium which flows to the right ventricle, but the output is swapped so the blood then flows from the right ventricle back out to the body without being oxygenated in the lungs. Blood returns from the lungs to the left atrium which flows to the left ventricle, but the output is swapped and the blood is pumped from the left ventricle right back to the lungs.

Fetuses grow more or less ok since they are getting oxygen from the mother. Upon birth a little bit of mixing of oxygenated and deoxygenated blood through the PFO and ductus arteriosus (and VSD in this case) gives newborns with TGA just enough oxygen to hold on until surgery. Surgical correction in the first day or so of life restores normal blood flow and is usually successful.
[/spoiler]

We understand TGA is very serious but there is about a 96%+ chance of favorable outcome. Plan needs to be in place prior to delivery. Patient has already traveled from hometown to major city in Colombia.

Mother has been evaluated at hospital #1 by a pediatric cardiologist and her doctor is saying he can’t do anything due to a narrow pulmonary artery.

Looking for any help, scientific study, etc… citing use of a graft or alternate approach that we could ask about when seeking second opinion. Know this is a long shot but there is another hospital with pediatric cardiologists in the city where she is being treated (Cali, Colombia). Making inquiries with Colombian Heart Foundation in Bogota as well. If, by miracle, there is another approach to pursue will probably go knocking on the doors of a charity to help cover costs not covered by insurance.

It’s worth trying for a second opinion before our family orders our second pint sized coffin (also a congenital heart defect) in less than 18 months. :frowning:

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Bumping my own thread once, for those who may stumble upon it for future edification.

First the happy news, after an incredible amount of stress, the mother has been sent home with specialists saying it was a series of four false positives and in reality nothing is wrong with the baby. Hard for me to believe that such errors could happen, but we are hopeful.

As to treatment of TGA with a narrowed pulmonary artery, here is what I found.

  1. Tissue engineering is not quite ready to grow a custom pulmonary artery in a lab and use it for transplant in a newborn. But it is oh soooo close. Had things turned out differently I would have been pounding on doors looking for emergency approval for a one off trial on humanitarian grounds. This is the state of research as of September 2017.

Such tissue engineering is in animal trials already and it seems such trials have been successful in sheep. Progress is slow but there has been at least one instance of similar technology in humans in a four year old. This case removed a small piece of vein from the patient, chemically stripped the cells away, and seeded with cells from the patient’s arterial walls. Graft was implanted and grew with the patient.

To get this approach to work in a newborn will basically require that a collagen framework is made in a lab, perhaps using 3d printing technology, and then seeded with cells from the baby. This makes an artery that is viable and can grow with the growing child. No immunological rejection either. The inability of an artificial graft to grow makes such grafts unsuitable for use in newborns, thus the urgency for a bio-engineered graft that can grow.
2) Stenting is less likely to be useful but might suffice in some cases. A stent is basically a mesh framework inserted into the blood vessel and then expanded in place to hold open the vessel. Stents are not normally manufactured in a small enough size to be useful in a newborn. And since stents cannot grow there may be a problem down the line in needing to remove a temporary stent.

However, a stent is just what was needed in the case of an 18 month old with a restricted pulmonary artery. The Los Angeles based cardiologist used a 3d printed model as a guide to manufacture a stent small enough for his patient. The child had a different congenital heart condition (Tetrology of Fallot) than TGA and had endured a few surgeries already. Having such an approach work in a TGA patient might be riskier but may be worth a try.
My final warning: I am not a cardiologist. I am not even a doctor. If you are reading this because your family is facing the impending birth of a baby with TGA then look to your medical professionals for the most up to date information. Ask for a second, third, or fourth opinion about the latest research before giving up. Real progress is being made.

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Happy to hear the great news, Iggy!