Plans Change.....

At least in our house they do!!
After Susannah's cardiologist visit in December we were waiting for a phone call to schedule a surgery date to have her PDA closed. Fairly simplistic procedure with possibly an overnight stay.
Last Thursday, I got a phone call from the cardiologist saying after he had met with the heart team and surgeons at the hospital, they were recommending doing a Pulmonary Artery Band (PAB) along with closing the PDA. Many questions ensued and a meeting put in place for this past Wednesday (which was the day of our regular appointment).
After almost a week of research, questions, pondering, praying, etc, the decision has been made to go ahead and have the PDA closed and the PA band done at the same time. We have a surgical consult schedule for late next week and will get to meet the surgeon.
That's the short version. :)

More explanation: Susannah has developed a leaky tricuspid valve and apparently it is concerning enough to the surgeons that they are recommending the Pulmonary Artery band to "train" her "left" ventricle to become stronger so that in a few months to a few years we will have the option to do a Double Switch Operation (DSO) vs. going straight to a heart transplant.
From what I have found from research and asking questions, etc, the leaky tricuspid valve is a significant indicator that the right ventricle is not doing as well as we would have hoped. Continuing on like this would mean at some point the right ventricle will most likely fail and it would be sooner rather than later, which would then put us at heart transplant. If we do the PA band and it works, we would have the option of doing a DSO. The PA band is found to work better the younger a person is when it is put in. It was at three months that they told us Susannah's valve was mild to moderately leaking, so in some ways I feel as if we're "behind" on the PA band. Some people say the optimum time for doing the DSO is 2-3 years old. It is not done on adults and some say the person has to be younger than 15. It will continue to be a wait and see approach and a careful analysis and watching from the cardiologist and surgeons. The "left" ventricle would have to be "trained" sufficiently before doing a DSO and I think it's wait and see how long that takes for Susannah. It sounds like everyone's different in how their heart responds and works. We're hoping that getting the PDA closed will help a significant amount, although the cardiologist didn't sound too sure that it would be enough, to help relieve some of the workload of Susannah's "right" ventricle. He also said having the PA band done may help the right ventricle as well because it would expand the Left ventricle and (in my words): push against the right side and help support it, thereby helping it be more efficient.
In my opinion it sounds like a lot of it is a guessing game as to what may or may not help, it depends on how Susannah's heart responds, but overall, we agree with the cardiologist and surgeons that this is a reasonable approach and the best decision regarding future options. (They also might go ahead and put in pacing wires for a pacemaker for when she needs one.)


Wednesday's regular stuff update: Susannah was in heart block again this whole visit. She did not gain any weight from three weeks ago - which greatly concerns him. He increased her Enalapril and increased her calories. He talked about the possibility of tube feeding - ick! I'm praying that after her surgery she'll really pick up eating. She acts like she could care less about eating. It doesn't seem that she's tiring out, she just quits and doesn't care, holds her mouth shut and flat out refuses! :)

Additional links and info (from an email I sent my Dad):

Susannah has Congenitally corrected transposition of the great arteries (also called L-TGA, or Levo-TGA) . It's different than Transposition of the great arteries (also known as D-TGA, or dextro-TGA). She also has Dextrocardia, which isn't a problem other than the idetifying right from left. She also has complete heart block, which can be caused from damage to the AV node during heart surgeries.  She also has a PDA(patent ductus arteriosus) that has gotten larger, and she also has developed a Leaky Tricuspid Valve.

They're talking do a PAB (Pulmonary artery band) to increase the work load of the "left" ventricle to "train" it for the option of doing a double switch operation (DSO) at some point.
These were some different articles and research information that I found that might make it more clear and be helpful.???... I put the year they were published beside them.

http://www.ncbi.nlm.nih.gov/pubmed/23820627     (2013)
http://www.ncbi.nlm.nih.gov/pubmed/12658191      (2003)
http://www.ncbi.nlm.nih.gov/pubmed/9755003       (1998)
http://eurheartj.oxfordjournals.org/content/18/9/1470    (1997)
http://ejcts.oxfordjournals.org/content/early/2012/05/02/ejcts.ezs118.full       (2012)

http://en.wikipedia.org/wiki/Levo-Transposition_of_the_great_arteries

http://www.chop.edu/service/cardiac-center/heart-conditions/congenitally-corrected-transposition-of-the-great-arteries.html#treatment
"Double switch operation. Surgeons create a conduit to shunt the blood from one side of the heart to the other. The heart's major arteries are also detached and reconnected. This corrects the circulation so that the heart's left side serves the body and the right side serves the lungs. This is a very complex operation and is usually performed in infants and children. The double switch procedure is very rarely used in adults."


http://www.achaheart.org/resources/for-patients/health-information/cctga.aspx
How common is CCTGA?
CCTGA is a rare heart defect. 0.5%-1% of all those with heart defects are estimated to have CCTGA. This means there are about 5,000 – 10,000 people in the United States with CCTGA. These are rough estimates based on birth rates.

Why a leaky tricuspid valve is a problem
People with CCTGA often develop leakage in their tricuspid valve. In CCTGA, this valve is located between the atria and the ventricle on the left side of the heart. This valve has to stay shut against the heart's strongest push out to the body. In a normal heart, this is the location of the mitral valve. The tricuspid valve is not designed for this additional workload. In addition, many people with CCTGA also have abnormalities in this tricuspid valve. This also makes this valve more prone to leakage. Over time, tricuspid valve leakage ("insufficiency") can grow worse. There are two possible reasons why this might occur. One is the excess workload. The other is that the ventricle itself is stretching and weakening. This stretching can cause the valve to leak. These two reasons can be connected, since too much workload can cause the ventricle to stretch and weaken.
In some people with CCTGA, replacement of the tricuspid valve is recommended. When done early, replacing this valve may improve symptoms and protect or restore heart function. However, valve replacement can only be done if the heart function is only mildly weakened. f the systemic right ventricle is too weak, valve replacement is no longer an option. When done late, tricuspid valve replacement has been linked to high mortality. Valve replacement in CCTGA should only be done at centers that specialize in ACHD surgery.

What are the long-term outcomes of the double-switch procedure?
The oldest people to have undergone a double-switch procedure are now in their twenties. This means we do not yet know what the long-term outcomes will be. Short-term results are encouraging. If you had a double-switch procedure as a child, you no longer have a systemic right ventricle. Many problems discussed in this section will not apply to your anatomy. However, you still are prone to the electrical problems discussed below. Surgery cannot repair the heart’s "wiring". The surgery itself may also cause additional problems. Surgical scarring can cause future rhythm problems. Conduits may become blocked and need re-operation. It is important to remember that the double-switch is not a "cure" for CCTGA. Everyone born with CCTGA should continue to receive regular care at a specialized adult congenital heart clinic.

http://www.achaheart.org/Portals/0/pdf/Library%20Education/CCTGA.pdf