Quick Surgery update

We heard from Dr. L today. The team at MUSC met yesterday and discussed Rebekah and her current test results. They have confirmed Dr. B's recommendations from heart Cath. Rebekah is going to have surgery to try and connect her non-working lung. We do not have a date yet. We should be talking with MUSC sometime next week about a surgery date.

D

Rebekah's Heart

It's finally here!  The diagram, with explanations, of Rebekah's heart!  :)  First, though, a diagram of a "normal" heart to give you a little perspective (thanks to Wikipedia for this easy-to-understand heart diagram!).

Okay, now this is the diagram of Rebekah's heart.  If you notice the cute little lacy, scalloped edges around the inside of Rebekah's right ventricle, it's not that way cause she's a cute and frilly little girl. :)  Actually, that means the wall of her heart is thickening due to her heart having to work extra hard to push against the high pressure in her pulmonary artery and lungs.  And I forgot to explain that the right sub-clavian artery is made of a dashed line because it actually travels behind the aorta and the superior vena cava.  We definitely have a beautifully unique little girl!

(If you are having a hard time reading all of my notes, click on the diagram and it should open larger in a new window.)

Nancy

HOME

We arrived home safe and sound this afternoon. Rebekah did great on the trip home and is resting well. We hope to get lots of rest in the next few days as things some what return to normal.

Thanks for all the prayers.

D

Where do I start.....

Rebekah came back from the cath lab shortly after noon today. As soon as we got the page that Rebekah was back, we knew right away that no procedures had been done. She was not in the lab long enough for any procedures. When we actually saw Rebekah, she looked really rough. She had a hard time coming out of the anesthesia today, and her oxygen sats had dropped into the 80's. So she was on a liter of oxygen through her cannula and 3 liters of blow-by (an oxygen mask is propped up by her face with oxygen turned on). When we first came in the room, Rebekah was moaning and whimpering in her sleep. She had gotten some fentanyl in the cath lab, and the nurses right away got her some Tylenol. After about an hour Rebekah was still moaning and crying, so the doctor authorized a dose of motrin and that seemed to be enough to take the edge off the pain and allow Rebekah to rest comfortably. Rebekah was also nauseous when she got back, so she had a dose of zofran as well.

Three hours (and one movie) later, Rebekah is doing very well. She is talking with us some, playing a little with her stuffed animals and drinking apple juice. She also had about half of a small container of strawberry ice cream (her favorite!).

Dr. Baker came in to talk to us after the procedure. We were correct in assuming that no procedures had been done in the lab. There were no areas of stenosis (narrowing) that needed to be ballooned or stented.

So there are two major areas that Dr. Baker discussed with us. First, the pressures in Rebekah's left lung have remained unchanged. That does not surprise either Drew or I. Her systemic pressure measured at 110 in the cath lab today. Her PA pressure measured 74 (this number is supposed to be 1/3 or at most 1/2 of her systemic pressure). Right now, Rebekah's PA pressure is about 3/4 of her systemic. So either the oxygen and medication have not helped at all, or the pressures have actually gone higher and the oxygen and medication are helping to keep them as "low" as they are now. Either way is not a good scenario. In spite of that, we are not going to directly address that issue right now, for reasons I'll discuss next.....

So way back to shortly after Rebekah was born, we learned that Rebekah did not have a right pulmonary artery. Rebekah's doctors had hoped at the time that Rebekah would grow some collateral arteries that could be tied together to create a right pulmonary artery to give her some use and function of her right lung. We waited and waited and checked, but nothing ever grew in a location that could be used to create a right pulmonary artery. Fast forward a couple of years to today. Dr. Baker found a nice, big fat juicy collateral artery that is supplying Rebekah's right lung with oxygenated blood. When I get home, I will scan and post a diagram so that you can all see what I'm talking about, but for now I'll try to paint a word picture. :) Rebekah's heart is very, very unique, so if you know something about the anatomy of a "normal" heart, just forget it for a second. :) Rebekah has an aorta (her former "trunk") and a pulmonary artery (her conduit) coming from the left and right ventricles of her heart. There is a left pulmonary artery branching off the main pulmonary artery (conduit), and there should be a right pulmonary artery, but it's missing. Off the aorta are the left subclavian artery (supplies blood to Rebekah's left shoulder/arm) and Rebekah's right subclavian arter (supplies blood to Rebekah's right shoulder/arm). Rebekah's right subclavian actually branches off to the left of her body and then winds it's way around to her right arm. I know, I know, she's very unique! Anyway, there is a collateral that has branched off of Rebekah's right subclavian artery that leads down to her right lung. It has always been there, but Dr. Baker said it has grown dramatically since her last cath. In fact, he said it has grown so much that the subclavian delivers almost no blood to Rebekah's right arm and almost all of it is going to Rebekah's right lung. Now that sounds good, but you have to remember that this blood already has oxygen in it. So Rebekah's heart is pumping blood through the left lung, back to the heart and about half of it goes to her body and about half goes to her right lung when it already has oxygen in it. This is a huge answer to Rebekah's fatigue problem! Her heart is doing double work to complete the job of a "normal" heart.

So, that's the problem, now what's the solution? Well, it looks like Rebekah will be having her third open heart surgery in a few weeks. Dr. Baker actually talked to Dr. Bradley, and they both feel that there is a good chance they can detach the subclavian artery from the aorta and reattach it to the pulmonary artery (conduit). There are some questions to be answered and much discussion to be had, but there is a good enough chance that they will be bringing Rebekah's case to conference in the next couple of weeks and putting Rebekah in "the next available surgery time." Wow. That is certainly not what we were expecting, but we are thrilled that she will be able to have at least limited use of her right lung. We were told that this will not make her lung function 100% normally, but even if it functions at 50%, it is much better than what she has now.

For now Rebekah will be discharged and we will head home and wait to hear from Rebekah's cardiologist about a final decision. From everything Dr. Baker said today, we anticipate being back in Charleston sometime in September. There is also the possibility that Dr. Bradley might upgrade Rebekah's conduit size if he feels that she has grown enough to handle a larger size (although she has not grown much since November). The reason is that he will already be in her heart, and if he can place a larger conduit, it might buy her more time before she needs another open heart surgery.

So that's the reason that the high pressure situation is going to be put on the back burner for now. Yes, it is significantly high, but Dr. Baker said it is worth doing the subclavian/collateral surgery first to see if that will help ease the pressure in the left lung by shunting some of the blood to the right lung. It is possible that it could significantly affect Rebekah's high pressures in a good way. If not, we can address the high pressure at a later date. I asked, and Dr. Baker said that Rebekah's hypertension could be managed as it is right now with oxygen and medications for decades before we have to make some other decisions regarding the hypertension. So Rebekah will still be on her current medications and the oxygen at least until after her next open heart surgery and quite likely after that. But he did say that we shouldn't allow the oxygen to interfere with her quality of life. If Rebekah wants to go to the park and it is impractical to drag the oxygen with her, then we should take it off for an hour or two. That was nice to hear! :)

I think that's pretty much a recap of the cath lab today. We are still in a bit of shock over the results, but are very excited about the prospect of Rebekah being able to use her right lung. Please begin to pray that the doctors will make the right decisions for Rebekah regarding her upcoming surgery.

Thank you so much for your prayers for our family! We have some exciting news that we will share soon, so stay tuned!

Nancy

Update #2

Rebekah is doing very well.  She has had sedated x-rays, an angiogram and at present the doctors are taking pressures in a collateral.  When we spoke with Dr. Baker this morning, he was a little undecided about what the exact course of the catheterization would be today.  Dr. Lucas (Rebekah's cardiologist in Greenville) has reason to suspect, based on Rebekah's echos, that there may be one or more collateral arteries feeding blood to her right lung.  A couple of years ago, that is exactly what we wanted!  In fact, we would love to hear that Rebekah has a couple of great big collaterals that can be used to make a right pulmonary artery.  However, the few collaterals that she had (prior to the procedure today) have been small and in very difficult locations.  They were not at all suitable for creating a pulmonary artery.

So the plan for today is to scope out any and all collaterals to assess whether or not any of them could work for a right pulmonary artery.  It is likely that Dr. Baker will simply take measurements and pressures of those collaterals today and will confer with Dr. Bradley (surgeon) to see if they can be used for a right PA.  If they can be used, Rebekah will at some point in the future have another open heart surgery to tie those collaterals together to form a pulmonary artery.  If they cannot be used because of size or location or complexity, we will probably be coming back in 3-6 months for another cath to coil off the unsuitable collaterals.  Dr. Baker apologized for the potential of another cath so soon, but we would rather that he is cautious before irreparably closing off any collaterals that might be used in the future to allow Rebekah's lung to function.

Another purpose of this cath is to check for any stenosis (narrowing) around Rebekah's conduit that might be ballooned open or have a stent inserted.  This would keep those arteries open and allow blood to flow freely, with less pressure.  Dr. Baker is also going to measure pressures in both ventricles of Rebekah's heart and in her pulmonary artery and left lung.  All of the doctors involved want a very detailed, clear picture of what is going on inside Rebekah's heart before they make any changes to her medications or oxygen.

So, I would guess that Rebekah will be out of the cath lab sometime between 1 and 2.  Then we will be in recovery for a few hours until she is either admitted to the floor or discharged.  If they do any kind of balloon/stent/coil procedure during the cath lab, we will be admitted for observation overnight.

Thank you for praying, and please continue to pray for wisdom for the doctors and safety for Rebekah!

Nancy