I just wanted to post an update on Nicole's surgery. She had her heart cath Friday, June 15, 2012  it was scheduled for noon but didn't go in until about 1:30 p.m. She did very well with the anxiety ... I knew how scared she was especially when we had to say goodbye as they took her to the cath lab.
The procedure took about 1 1/2 - 2 hours so Rick and I went to the cafeteria for lunch and then waited in the waiting area. They called us twice to let us know how she was doing. The doctor's came in after her procedure. The cardiologist who did the procedure said that her heart looked fine and that her holes were pronounced and everything from that standpoint looked as expected.
The other doctor (her PH specialist) talked about her lungs. He said her pressures in her lungs are the equivalent of her systolic blood pressure but I'm not sure if he means the normal bp or her bp but they are extremely high as the pressure in your lungs should be no more than 25. This is as it was 2 1/2 years ago. He isn't happy with the results of the inhaled drug (Tyvaso)  that she has been taking for the last year and a half which has the same medicine that she will be switching too but at a much lower dose. Nicole will be switching medication (Remodulin)  and she will be put on continuous medication via a subcutaneous (SubQ) sight as the IV/PICC line route has more risks with air getting in the line which is deadly for someone with a hole in the heart. This medication is the last step and once this stops working there will be no other options for her except for a lung transplant unless they come out with some new more powerful drugs or there's a cure.
I am nervous about switching as I will have to learn all about a new medicine and be trained by the drug manufacturer as I did with her inhaled. I am concerned as the sub cutaneous method which supplies continuous medicine through the skin is very painful at the site and I don't want to see Nicole in pain. She is too scared at this point of the IV. At the hospital her anxiety was extremely high when they had to put the IV in her hand. I had to hold her other hand and keep her still. The switch over should happen the end of July or beginning of August. Fortunately, the SubQ doesn't require a hospital visit only a doctor's visit to start. The only issue that the doctor is concerned with is the fact that Nicole is so thin. 
I was disappointed with the results but they were what I expected. They have told us from the beginning that her pressures will not come down with the hole in the heart but the hole can't be corrected because of her pressures. It is a catch 22. I am extremely grateful and I don't think I really realized this until Friday  that even though it was gross negligence on behave of the doctor's etc. that Nicole's congenital heart defect wasn't diagnosed it is a true blessing because it is because of that defect that has kept the PH from progressing like it would normally. If it wasn't for the heart defect we would have probably  lost her already and for that I am extremely grateful.
We got home about 9:30 a.m. Saturday  morning. I spent the night with Nicole in her room. She didn't get much more than a hours worth of sleep and I may have gotten a couple more than that. They woke us up at 4:30 a.m. and kicked me out of the room to take a chest xray in her bed which was required for discharge. Nicole didn't get back to sleep after this and was only the first or second time in her life that she has seen the sun come up.
Going forward I will pray that while the new medicine may not lower her pressures I will have to rely on it improving her quality of life which is a statement that I dislike very much. I will be happy and grateful if she has more energy and isn't as fatigued all the time. I pray that she will tolerate the site pain without pain killers and she will tolerate the medicine with the least amount of side effects. The PH specialist told Nicole at her initial appointment in Feb. that he believed that he could make her feel better.  He asked her what her top three wishes were in regards to her illness and the top one was her  fatigue level. I told him Friday that Nicole and I were going to hold him to that. He laughed and said "oh boy, I'm in trouble now".
Thank you for your thoughts, prayers, emails, facebook messages etc. I can't tell you how grateful I am.