Saturday, September 20, 2008

Hanley Conference

Yesterday evening, we spoke with Dr Hanley about Wren's case. He was very reassuring and we were impressed by his experience, clarity and willingness to explore the benefits of alternative surgical paths and entertain changing what he does. After speaking with him we feel that he would take on our concerns about the Ross and still do the best for Wren. I mentioned that our second cardiologist may call him to discuss Wren's case further and said he would be happy to do so.

Here are a few of the main points which we found so reassuring:

1) Dr Hanley has performed 100's of sub-aortic resections. He has done only 12 modified kono's (a year?) because he feels that he is almost always able to get at the obstruction through the valve. "The hemi-kono is more of a destructive operation. You are making a full thickness cut of ventricular septum and there is more chance of disrupting the electical impulses to the heart". He says he expects to perform a myectomy on Wren and that you can get an effective resection either way.

2) He feels confident he could get enough of the sub-aortic obstruction via the valve. If the valve was too restrictive he would move to a modified kono (although he dislikes this terminology and calls it a hemi-kono). He describes Wren's sub-aortic obstruction as "moderate" severity - a ridge/tunnel combination.

3) Dr Hanley will be very aggressive in saving the valve. Even if Wren is left with mild to moderate obstruction after the surgery he feels that would be worth it if he believes Wren will get 5 years out of the valve. "We have looked a the pictures and my guess is that with relieving the blockage under the valve and supra valvar blockage the valve is not going to be perfect but likely it will be sufficient."

4) Regarding the Ross-Kono: "I personally don't do Ross procedure unless it is really in child's best interest. I look at it as something of a temporizing maneuver because there are no 'good' options, esp since you are putting prosthetic on right side." With a Ross you are buying lifelong procedures one way or another - surgery at age 2 and then probably conduit replacement at age 7 or cath at 7. Then at age 12 or 13 you still need a surgical conduit and then they have a 15 year lifespan.

5) If we don't want a Ross done: An alternative to the Ross is the formal- Kono. Then we would put in a mechanical valve or a bio-prosthetic. Now, you might only get a few years out of a bio-prosthetic valve - but there are some complications with the bio-prosthetic valve because these valves are more bulky than the mechanical valves. The mechanical are more streamlined and could last till puberty... but you are on Warfarin. I ask my families "are you a Bobby Fisher or Evil Knevil" (sp!) and the choice of valve is really a matter of philosophy - not a technical consideration."

After speaking with Dr Hanley we are reassured that we will be able to find a good and workable solution. We were most impressed by the way Dr Hanley was comfortable with many variables and shifting parameters.

Right now, we are leaning towards going to Stanford and have the following list of 'surgical options' in mind. The replacement valve selection is still tentative and we need more information - hopefully from the cardiologist we consulted yesterday:

1) [IF VALVE APPEARS SUFFICIENT] Save the valve with myectomy.
2) [IF VALVE ACCESS RESTRICTIVE BUT APPEARS SUFFICIENT] Retain the valve but perform aortic resection via hemi-kono.
3) [IF VALVE INADEQUATE BUT LARGER VALVE CAN BE FITTED] Perform Kono procedure with bio-prosthetic.
4) [If VALVE INADEQUATE AND SPACE SMALL] Perform Kono procedure with mechanical valve.
5) [IF NONE LOOK GOOD SOLUTIONS:] Ross-kono procedure.

We think this is the best path for Wren but we are still unsure of the valve replacement priority in options 3 --> 5.

I am now in action mode, contacting all the parties at Stanford to arrange accommodation, pre-op appointments, Mum's flights [she is doing this but we are talking dates], child-life etc.

3 comments:

Heather C. said...

Shannon,
I'm so glad to read about your progress in these two recent posts. I completely relate to your impressions of Dr. Hanley (and I hope that even more insight comes from Dr. K's research and outreach). Would love to talk more if you want to go to the park some time. I was in Palo Alto (alone) the month before Evan was born (at the Residence Inn) and we finally moved into RMH for the month following his surgery/recovery.
Hugs to you and Wren and family.

Shannon said...

Thanks Heather
We had another momentum stopping conversation with Dr Lewin this evening. He seems sincerely concerned by the risk of leaving an obstruction doing a myectomy instead of hemi-kono.

I am extremely tired.

I suggested he speak with Dr Krabill and that perhaps they could agree on a strategy.

Heather C. said...

Ack. Can Lewin speak with Hanley directly about his concerns? That would be a reasonable expectation, no? And then synthesize their points of convergence/divergence for you? Is Dr. Silverman the cardiologist that reviewed Wren's case with Hanley? It seems he would be knowledgable re: concerns about the myectomy. I'll be anxious to learn how Dr. Krabill comes down on it all.

You are showing remarkable strength and clarity here.