Short version: We met with Dr. Kuperschmid yesterday. Maya will have surgery Methodist Hospital in San Antonio to repair her and most likely she will be fine with that until she is 20 or 25 years old when they will have to replace a ring around the valve as she outgrows it. Worst case scenario is a mechanical valve that would make her life different from ours.at
They will give her a liquid Versed to calm her before either Brandy or I take her to the OR and they will give her gas so they can insert the IVs, transesophageal echocardiogram tube, ventilator tube, stomach tube, catheter, and arterial lines for the bypass.
He said the riskiest part of the surgery is actually the sternotomy—entering the chest with the heart pressed against the breastbone. If the saw hits the heart, he said it “is a bloody mess for a while.” This has happened twice in his 20 years or surgery, so chances are that this happens are almost 0.
After that, he said they would put her on heart bypass and inject the heart to stop it. Then, they will enter sort of where they did before on the upper left (facing her) side of the heart wall, then cut through the atrial wall, and work on the valve from that side. I was expecting them to enter on the right side as this is where the valve is. When I asked him how big a hole this is to work through, he held his fingers about an inch and a half apart. So, on the attached pic, they will enter on the upper left atrium, go through the wall, and work downward on the right valve: that white thing with all the strings hanging down from it on the right side.
The valve itself sits in a muscle ring called the annulus—where the white valve connects at the top. Maya’s is not the shape it should be so they will stitch in a ring called a Carpentier ring to fix that. The flaps in the valve are tethered down to the lower part of the ventricle walls by things that look like fine strings and the closest one on the pic has strings that are too short on Maya’s, leaving it unable to close completely enough. He said he would just cut the ones that are restricting it and that should allow that side to close better. The other valve flap (the back one in the pic that you can’t really see) has an apparent cleft in it which will either be stitched in a manner so that it closes better or resected and rebuilt out of surrounding redundant tissue.
They will then close the atrium and heart wall, allow the heart to restart and look to see how much regurge (leakage back up into the atrium when the ventricle contracts) is showing on the echocardiogram. If it is good enough, they will close her up and get her off the ventilator as soon as she can breathe on her own. He said that if things go well the surgery will take about four hours. It doesn’t look good, they will re-open the heart and try again.
He said he was 75% sure they could repair this as described above, but even with the pictures they took most recently he was unsure if he knew exactly what he would find once inside. If the valve cannot be repaired to his satisfaction, they will insert a mechanical valve. If this happens, Maya’s life will be significantly altered because she will have to take blood thinners for the rest of her life. This means anything that makes a bleed (internal or external) is far more dangerous to her than a normal person and she would be unable to have children of her own because of the effects those drugs (Coumadin/Warfarin) have on young girls. The valve will also have to be replaced at times.
If everything goes to plan, the insertion of the ring on the annulus remains a small problem and will begin to be too small for her at some point. He said probably around age 20 or 25 she would have to have this surgery again. By that time, though, it should be relatively easily done because of her size and the technological advances.
Personally, it helps me to go through all of this to sort of callous me a little and dampen my fears by pretending that understanding is controlling. Brandy is taking it pretty hard, and the prospect of the mechanical valve really has her worried.
Your thoughts and prayers are appreciated.