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.
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.