We meet with Dr. Pharis this morning to talk about the procedure. What they were looking for and sign the consent form. It was nerve wrecking as Dr. Pharis was hoping that if they found this stenosis he hoped that it wasn't total stenosed veins as if they were there was absolutely nothing they would be able to do for them as it they were total stenosis 'ed they would be small from tip to tip and the lung side goes deep into the lungs where they can't fix as the lung is very spongy delicate tissue. The procedure was to take approximately 2 1/2 - 3 hours long.
They needed to sedate and paralyze her for the procedure. So we waited on pins and needles as the hours went by. Finally a little over 4 hours later They came in to tell us that she was on her way back up and that it went well.
We sat down with Dr. Kakadekar and he explained that they were able to get everywhere they wanted to except for one pulmonary vein (top right). She did need a transfusion of 60 cc. Which apparently is quite a bit for a little one. He said however that her stenosis is very small, but that they where able to tell that her lower right pulmonary vein had formed to the right atrium instead of the left where it should be. The PDA was still very large, she also has what they think is a medium/large ASD (however it looks to be shunting the blood flow in the wrong direction), really high pressures in her lungs and pulmonary artery. They were fairly certain however that the "shelf" in her aorta (coarctation of aorta) isn't causing any major problems.
Because of the fact that they weren't able to see the top right pulmonary vein and where it was going to, they wanted to do a CT scan to reconstruct her heart and confirm the cath results.
It was a huge relief as he was very convincing that it all sounded fixable, and she was going to be fine.
Angiogram
Pulmonary Vein Stenosis
Coarctation of Aorta
ASD
PDA
TAPVR/ PAPVR
Pulmonary Hypertension
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