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.

Timing
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.

This adds 20lbs

Wren is coming OUT! They did not do any ballooning. The obstruction is under the valve so that indicates OHS. Crap but what we expected.

Here I am dressed for surgery.


Here are Josh and Wren on the Twin Falls hike on the weekend.

Wren is in surgery

We are waiting in the Family Resource Center at Childrens. Wren went into the cath OR at 8am. The procedure should take 2 hours with 1 hour being the cath intervention and 1 hour going in and out of general anesthesia.

They allowed me to walk him into the OR and hold him until he was sedated with gas. I picked Root Beer flavor but when I kissed him bye bye the gas did not smell like root beer to me.

Nurse Amy from CICU is our nurse today. It was great to see a familiar face although everyone is a bit familiar after our last dry run.

This morning we spoke to Dr Jones regarding the likelihood of his doing the ballooning of the valve and arch. He seemed more circumspect about doing it that I had expected. He said that they will only intervene if the valve or coarc are the dominant factor in the LVOT and arch gradient. There are expected to be many components but it is not considered worth the risk of a ballooning for a minimal improvement.

He is going to call us during the procedure to let us know whether they are going ahead with a ballooning. If not, it should indicate that the sub-aortic area is the major issue.

They are also going to see whether the mitral valve is involved (terror in my heart). Surely, I hope not. The echo has not shown trouble in the past but I guess we need that confirmed.

More soon.

Follow-up to Children's last Friday

Over the weekend I submitted "Feedback" about our bad experience being bumped last Friday. I tried to make it constructive and pointed to the things that were a particular problem:

1) The fact it happened.
2) The last minute notice.
3) The lack of empathy (from everyone except the Cardiology Social Worker) and,
4) The feeling of being left without any 'advocate' in our medical team.

Today, I received a call from the Director of the Children's Heart Center - Joni Blanchard (??). She had a very easy manner and I felt she was looking for ways to do things better. She said that she had investigated what happened on Friday and it was a combination of issues. In addition to the Hospital running with little capacity, I learned that the main problem was that Dr Johnston did not request a bed when he booked Wren's cath. She did not know why he did not do so (perhaps he did not read the details enough to know the high odds of requiring an overnight stay or it could have been an error). Regardless, a bed can and should be booked beforehand despite what he told us to the contrary.

On this point, apparently our bed booking for August 7th is "contingent". This means we are on a waitlist for a solid booking and could have Wren's bed booking bumped for higher priority cases. However, on Tuesday 5th there is a "solid" bed booking available at a time Dr Jones has an opening. I am waiting to hear from Briana [surgery scheduler] whether this is first case or something later - we will have to make a decision then but a confirmed bed sounds less risky for a repeat bump.

I also asked about the continuity of care and advocacy for Wren. I wanted to know who was our point person on the Children's Staff. When you have a cardiologist it feels like you have a primary relationship with them because of the continuity of treatment. I feel that Dr Lewin is like Wren's heart care manager but perhaps they don't perceive it in that way. Perhaps they feel that the whole Heart Center works together. From a patient perspective, it seemed that once Wren was referred for cath, the cath interventionists became his entire medical team and existed in a vacuum from other areas of the hospital. I suggested to Joni B that we would like to know who is primarily looking out for Wren beside us. For example, I would have appreciated a call from our nurse at the Children's Heart Center or from Dr Lewin (who is on vacation this week it turns out). If 'our' nurse had called and said "I will make sure Dr Lewin knows what is going on" or he had called us at a later point that would have left me feeling that they 'cared' or at least was overseeing things and making sure that their patient was treated with appropriate priority.

We now have a confirmed reschedule:

Tuesday 5th August at 6.15 am (first case on early start day is 7.30am)