Consult with Surgeon #1

Today we met [face to face] with the Seattle Surgeon from whom we received our first surgical opinion. I expected it to be a routine, 10 minute chat in which we asked about experience, volume and outcomes and then said "see you later".

This was not the case.

I feel a sort of insane hilarity in writing this but it seems that either the surgical plan was incorrectly communicated in prior conversations with our cardiologist OR it has changed. Regardless, there were some significantly different elements to The Plan as we learned of it today. It was a very useful and informative meeting.

The Crib Notes Version of the Consult
Plan A:
Save the Valve
Open chest. Inspect aortic valve. IF valve appears acceptable perform a modified-Kono procedure to enlarge the aortic root and sub-valvar area and remove the obstructive tissue.

IF Valve appears floppy or patently insufficient after supportive sub-aortic tissue removed -----> PLAN B (valve replacement).

Perform a myectomy through the aortic valve to remove any remaining obstruction.

IF Valve OK: Off bypass. Observe valve performance.

IF gradient is satisfactory, valve motion acceptable, minimal regurg ---> Close.

IF gradient unsatisfactory ----> PLAN B (valve replacement).

Plan B:
Aortic valve replacement with mechanical valve and aortic root enlargement
Take down the modified kono [remove patches]. Insert single large patch across valve. Insert new mechanical valve. Off bypass. Close.

Start lifelong warfarin treatment.

------------ END CRIB NOTES -------------------

DISCUSSION FOR THE CHD INSIDERS or WANNABES or STUBBORNLY TRYING TO UNDERSTAND (like us)
Why not do a Ross-Kono procedure?

The R-K gained a lot of popularity in the 90's so we have some pretty good series data at this point. The data shows that over ten years the procedure has a 20% failure rate of the autograft in the aortic position. This is the average failure rate. We know that there is a higher failure rate in patients with risk factors of BAV and Coarctation. They are indicators of a Fibrillin disorder (a connective tissue disorder) which leads to a higher failure rate. Now, I don't know the rate offhand but even if we say that those patients are the 20% - that is too high. ..... I have three boys and if one of them had this condition I would not do a R-K procedure...

Another concern is sensitization/ development of antibodies due to the use of human donor tissue for the pulmonary graft. We try to keep options open in young children and we are having lots of problems in cases where children eventually need life saving heart transplant surgery because they have antibodies due to these grafts. This makes it very hard to find a good tissue match. In the US, bovine grafts are not FDA approved for use in initial pulmonary valve replacements but are approved for subsequent valve replacements. That means we would have to use a human tissue graft for a Ross-Kono pulmonary replacement.

Look, the R-K is a surgeon's procedure. Surgeons like it. Its about opening the whole heart up, fixing it and putting it back together. Its a great surgery - I enjoy doing it but I don't think its the right surgery in this situation.

SAM - Systolic Anterior Motion
A risk in cases of complex LVOT is SAM p where one of the mitral valve leaflets gets pulled into the valve due to the venturi effect. This leads to a significant obstruction. A modified Kono would open up the area and overcomes risk of this complication.

Heart Block
All the Kono procedures are high-risk for heart block. We see about 15% of patients develop heart block. Interestingly we have had a couple develop late onset (2 weeks out) heart block. In most cases it occurs post-op. Children then need to be fitted with a pacemaker. Its risky because the place where we make the incision is the electrical center of the heart but it is impossible to know exactly where it is so it is sometimes cut or damaged by scar tissue. We do our best to follow indications about position but its just a high risk in this procedure.

What about the bio-prosthetic valve we were expecting?
These Bio-p valves do not last long in young children with their fast metabolisms. Its not about outgrowing the valve in size but it degenerates. You would be lucky not to be back inside 5 years. We see it all the time. I would hope to get in a small-adult sized valve but he would need Coumadin therapy with it. I would have to see how big the valve could be but if he grows to his father's size (5 7") he may not need another valve for size - if we can get in a 21mm valve. I will just do my best but you can't be sure till you see it. Sometimes a large valve will impede the motion of the pulmonary valve and some kids can tolerate that and do very well but we just have to see.


Kono-procedure complicates subsequent valve replacements
A Kono procedure also makes it difficult to do valve replacements in future. Many surgeons can't/won't do it. Its a tricky surgery. You have to take down the Kono and resize and reshape the area with new patches [I didn't really follow the detailed explanation of the steps in the take-down and redo. Frankly, anything with Take-Down in it freaks me].

In usual Modified-Kono a L-shaped incision is made but in order to enable a translation to a Kono and Valve replacement in future he would use a straight incision that is appropriate to both surgeries. Also, he would use some techniques that would allow a Ross to be done in future [I may have this wrong].

Surgical experience/outcomes
The facility should do over 400 OHS this year. They are classed as a moderate sized institution. I have never lost a patient doing a Kono procedure but I can't promise that in writing. I do about 3 a year - not because of the complexity but because of the rarity of this condition in children. I have done about 40 of this procedure and related Ross procedures.

Next Steps
I have placed a call to Dr Lewin to talk about a lifetime on Coumadin/Warfarin. I am also interested in the issues of serial valve replacements with the Kono-take-down issues.

Meeting Dr K, 1pm Thursday.

Phone consult with Hanley, 9.30am Thursday.

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.