Tuesday, August 12, 2008

Surgical Consult #1

Cardiology Conference Surgery Plan
This morning I spoke with Dr Lewin about the discussion of Wren's case at Children's cardiology conference.

Everybody agreed something had to be done about the outflow tract obstructions so the question was what operation to perform.

Factors in the Decision - Valve, proximity, the unknown
The decision of what surgery is best is difficult because while the sub and supra valvular obstructions are well defined we have no idea how abnormal the valve is and how obstructive that valve is. We know it is bicuspid which compromises its operation, we know the leaflets are thickened. We could find there is an obstruction sitting in the background.

Also, the aortic valve is on the small side (mildly stenotic). Wren's is 8mm at the valve while normal range for his size is 10-14mm.

Next, people were very concerned about how close the subaortic 'tunnel' comes to the valve. If it is further away from the valve you can carve away obstructive muscle but when the muscle comes right up to the valve that is impossible. BUT if you don't cross the valve and get the muscle where the leaflets attach you get obstruction after the surgery because you are leaving that muscle behind. Now, you don't want to find you that you have to do it again soon but you don't want to do something to a valve if you don't have to with young child [note: because of valve replacements, issues with artificial valves, need for re-operation when valve outgrown or degenerates].

So, what do you do to have best of outcome with as little morbidity [aka Death] as you can get? We get to balance the risk of operation versus outcome.

In this case much of that is a "gut check" decision - not data driven.

Surgical Options
The group discussed surgical options:
A) Patch above, evaluate valve, carve below.
B) Patch above, valve replacement, carve below.

By the end of the discussion everyone was in agreement that they should REPLACE the aortic valve - option B. These would be the various repairs:

1) PATCH Above the valve. A Y-shaped piece of pericardium or gortex would be used to patch the aorta where the obstruction is removed. Very quick straightforward procedure.

2) Outflow tract - KONO procedure. They would take an incision through the valve and replace the valve. There are three types of valve used as replacements:

  • Mechanical valve. A mechanical valve should last till adulthood in right size. Would then require replacement. Recipients have to be on Coumadin [why, think this sucks, why so unpopular?].

  • Animal valve on metal scaffold. No coumadin required but animal valve degenerates and needs replacement sooner. Estimate: 5-10 yrs

  • Ross-kono: Replace aortic valve with patients own pulmonary valve. This is generally surgery of choice for young children but is not indicated as well in cases of bicuspid aortic valve. Studies in past 5-10 years show that Bicuspid AV is indicative of a connective tissue disorder that does not cause the patient problems but is correlated with a higher rate of degeneration of the replaced valve [ie the pulmonary valve acting as the AV would degenerate]. Time frame for degeneration is similar to the animal valve PLUS you have messed with the PV.

They have not determined which valve to recommend but I got the vibe that the Mechanical with Coumadin was leading slightly.

What about the Referral to Hanley?
Dr L said he was interested to hear the recommendation from Stanford and that they might say "the Ross works great". He said that he was sending the packet today and has spoken with Dr H's staff who promise to bring the case to conference this week. He expects to speak with the surgeon or presenting cardiologist by the end of the week but if he has not had a response by Tuesday he will call again.

The feeling was that Wren is not in danger on a daily basis so surgery is not indicated in the next 2 weeks but a timeframe of 1-2 months, under 3 months is recommended.

Risk and Choosing where to go
I asked when to have the discussion about the relative outcomes at different centers or what the major risks were. He said that once we hear back from Stanford we will sit down with him and talk through the risks and issues in detail. He did say that the valve replacement made it "a big, major surgery" [I guess not all heart surgery is big?]. He said that at that point he will put us in touch with Dr Hanley to talk about outcomes and risks there too.

Odds and Ends
This valve replacement makes sense to me after talking it through but I am still in shock. Why does every step we take leave me in shock again? Immediately after the conversation with Dr L I went to Wooly Mammoth and bought TWO pairs of shoes. I am usually unable to buy shoes but apparently shock and dread make me decisive. I just bought them and they are both great.

For some reason I am particularly apprehensive about Coumadin. My baby is going to have MAJOR, BIG heart surgery and I am freaking out about a drug. I suppose its the never ending ness of Coumadin and the feeling of being poisoned continually. I guess I am overreacting? Right? You can tell me I am over-reacting but I just want him to come out of this not to be sad and sick forever.

Of course, my mind is also spinning out frightful scenarios in which Wren dies in surgery and I am wearing my new shoes. I think "are these the shoes I will be looking at?" like some crazy lady in a novel focusing on her hairdo while the world burns. I think, we shouldn't go to Stanford because we can't have a funeral down there! We don't know anyone. I saw an interview with an Olympic Gymnast last night and she was asked about her blog in which she shared a recurring thought that she would fall in a move on the uneven bars. THe post had fans and family in high anxiety. She said "that is just a way of working out my nerves, I mean, I didn't fall did I?" I liked that. My dreadful fantasies of shoes, funerals in hostile California and of having to counsel Frost forever are just my working out of Olympic Heart Surgery jitters.

You get to say YES here.


flora said...

Hi! I made my way to your blog b/c I'm on the bbc site too (fshnyc). My son has TGA and had his switch in February and now we're facing surgery again b/c he's developed coarctation (it was debated at birth but not bad enough to operate on). I'm going through the same thing- it seems like everytime I talk to our cardiologist and get on board with whatever he's shocked me with at the appointment- we get something NEW to shock and traumatize me. Totally get it. I know you didn't ask and its none of my business but you could consult w/Stanford yourself- that way you'd be able to ask Dr. Hanley everything yourself- and usually the consult is only a few hundred out of pocket (and 0 if insurance pays). I've heard amazing things about Dr. Hanley- he is one of the world class congenital guys and given the situation- I'm sure he's he best guy out your way to handle Wren's little heart. Best of luck and I'll be reading to check on him!

Valerie said...

Alexia chose the St. Jude Mechanical for the specific reason that she would not have to go under the knife for 25/30 years. Her other valves only lasted 7 years. Coumdin has not been an issue for us, it is the only med she is on and very easy to regulate. They d have home monitors now.

FYI, Stanford has an ridiculously high infection rate, although they have the best surgeon in the world. It is kind of a hard decision. Too many hands in kettle, if you know what I mean.

If I can help you any more, please don't hesitate to write.

Shannon said...

Hi Flora
I am so sorry to hear about your son's coarc. After a switch its so hard to hear about something else. Wren had a coarc repair at birth and it was relatively uncomplicated and without bypass so I hope that you have a similar experience if you son needs surgery.

Do you have a blog? How old is your son?

I think we will wait for the consult because it is in the works already.

Valerie: I am scared about that infection rate. I know that Drew had a post-surgical infection but I hadn't realized it was common. I shall inquire further. I still feel it is better to be with the best surgeon for a complex case like this but it is something to consider. Is there anything we can do to minimize the risk or is it from the actual surgery, I wonder.

Valerie said...

My husband, who is a PICU Heart RN, says to give Wren a surgical hand soap bath before surgery. They use to do that before and then stopped for some reason. But he said it was quite beneficial.

Lindsay said...

Gosh Shannon. It feels like I am reading all about Marin.
They have the most similar problems. I can't believe that it is the same thing that Marin is having problems with at the moment. Who knows maybe we will be at Stanford at the same time?

marin had the patch placed above her aortic valve in her 1st surgery and it has no longer been a problem. It seems to me like it might be better to work on the outflow tract first, if attainable, rather than shoot for the Kono. I believe that usually they tend to do the Kono on younger children before they automatically suggest a mechanical. Who knows though it seems like there are so many options.
Marin also has the bicuspid valve as well as mild regurgitation right now.
Does Wren seem like his activity levels have lessened?
Marin is sure seeming like she is calmer lately. I am beyond scared and calling her cardiologist in the morning.
I can totally relate with your feelings. I am scared, sad, anxious, mad, and tired of feeling like we will never know what the future will be. I don't envy parents whose children have to have the 3 step process, (Norwood, etc.) but I do envy the fact that they typically know what and when to expect things. I try not to think this but it is hard to never know when Marin will require surgery again. We have definitely been shocked with this one.

Izabell said...

Shannon if I am remembering right they gave Izzy a bath with special soap right before her heart surgery, maybe because she went from the CICU for her surgery because (she was admitted to ICU a week before her open heart so she did not enter to surgery from home) i don't know if they do that prior at Seattle from the surgery center though? Maybe something to ask?

As for all those "thoughts" Steven always said that my head played the meanest games on me. I had the worst thoughts and dreams while Izzy was facing surgery and in recovery. I hated thinking those thoughts did not want to but they came with my fear. I am so sorry that you are having these thoughts I have asked some other heart moms and I think soo many have these fears and thoughts. It is not like you sit there trying to think them up it is more like they pop into your head and your shocked by them! Then you try to think them away, reason them away. I felt awful but I think it is just our fears coming thought when we are trying so hard to be brave. Any thought that comes though your mind is there because you love him and it is a very frightening time. I will be sending all our love and thoughts thought out and will share any info I get with you. ~wyndi

Jennifer said...

I'm so sorry to hear about little Wren.

Being with the best surgeon was priority on my list, too when I chose Dr. Starnes at Children's in LA for Justin's surgical repairs. However, his cardiology as well as other teams are all in Orange County. I do feel there is a loss of translation along the way, like Justin's care is not as seamless and well understood as it would be if I were to have all his care in one place. It is not uncommon, however, for families to choose one institution for surgery, and another for routine care. This is one of the nice things about having private insurance. It is a pain to deal with the insurance and meeting a whole new team of people who do not necessarily know your child as well as your cardiology team, but the surgeries are important. It seems Wren has a complex case on his hands, and you would want someone with the best experience to care for him. I think you're good at following your instincts, and you should do what feels right. Meeting the surgeon is a good idea, too, so that you get a feel for the guy.

In addition to Valerie's response, Justin also got a full body antibacterial Dial soap washdown the night before his surgery and no one was allowed to touch him. The surgeon wanted the soap to be the last thing that touched his skin.Pretty good precaution.

I can also relate to those feelings of shock. It's like we know that life will be so uncertain, yet when the uncertainty reveals itself we are so completely surprised. I think in these situations, we're always preparing ourselves for the worse case scenario so that if something happens we think we won't be as shocked and we'll be prepared for it. But, we never are. I say we, but I think I mean I.

Praying for you and little Wren.

Shannon said...

I am also surprised by the similarity between Marin and Wren's heart issues. I haven't heard of any other kids with supra-valular issues. It is supposed to be particularly uncommon.

We haven't seen regurgitation with Wren (yet) but the cardiologists seem to think that valve behavior is affected by the SAS which is why they are so cautious about 'saving' the valve. They think that the chance of re-operation to resolve it is high.

Thankfully we have not seen ANY change in Wren's activity. He is very wild and active with his brother over summer. He runs and jumps everwhere BUT he does grunt a bit and breathes audibly with exertion (no sweating though).

I am sorry you are seeing some changes in Marin's energy levels. Of course, it could just be a something else intangible but I know the feeling of dread. I think I have told 4 doctors/nurses that Wren grunts when he squats and half of them think he is pooping. Nope. He always grunts in certain positions.

I follow your blog and will watch and see what your plans are with Stanford.

Dina said...

I too have thoughts like you are having. Totally normal, I like to think.
Don't have any words of wisdom to calm your fears except these heart kids are amazing.
I will keep you and Wren in my thoughts and send positive energy your way.