Our consultation with Dr Lewin was relatively brief for the bad news. Apparently, the level of turbulence through the aortic valve has increased significantly and we are now on the surgical path.
Since the velocity was so high it was not possible to distinguish the obstruction. Dr Lewin believes it is a sub-aortic muscular bulge which is producing a diffuse narrowing in the sub-aortic area (SAS or Sub-aortic stenosis). This is not a membranous ridge as I first thought.
The plan is to do a cath within the next two months. The cath should show where the stenosis is critical - SAS, valve or above the valve. If it is the valve (he thinks this unlikely) they would try and balloon it immediately. More likely they would refer the findings to Children's Hospital cardiology conference and see what surgical approach they recommend. The options discussed were "cutting away" the ridge or removing that section of the septum and replacing it with a patch. The removal of the ridge is preferred but is not always possible due to access down the ascending aorta and it is a complicated diagnosis due to rarity and the high rate of recurrence after surgery (he did not mention that this time but I know about it due to other kids with SAS).
Here is an article about it.
I am feeling flat and anxious. I hate to be in this place and worry about Wren now facing open heart surgery when he looks so well and active.
We discussed the best place for surgery and Dr Lewin basically said we had to do the research. I said we would consider Stanford and he said that because we have the luxury of time, after the surgical recommendations come through from Seattle Children's Conference we could then send the findings over to Stanford for a second opinion and to see what course they would suggest.
I do not want Wren to undergo surgery at all if its not necessary but if it is I hope we can get through this before RSV and flu season. It sounds as if the timing is definitely before the end of the year.
6 comments:
This is not great news :( I didn't realize you were so close to surgery (perhaps you didn't either), Wren seems so hale and hearty.
Does this new info surprise you? I know before Ramona's echoes I'm always convinced that everything's fine because she's doing so well. I supposed it's good that they find problems and address them before they get really sick, but it is SO hard to send a smiling, seemingly healthy baby off to surgery.
You guys are at the top of my thoughts tonite and in the days to come...
I knew that there was a strong possibility of this surgery at some point. With Shone's the obstructions tend to be "progressive" meaning that they get worse over time. However, progression can be very slow and it can be stable (ie no change) which is how we were last time.
I guess I hoped it would be when he was older - like 4 years old. Not yet. I have this feeling that pediatric cardiology is developing at such a pace that any delay is good in that it opens up new possibilities in treatment and standard of care.
My mother also said that its good they found it now. Like you said, its so hard to believe its necessary when he looks so strong and 'normal' in his activity levels.
i am sorry that this news as come and and hoping that the cath lab will help delay the need for surgery. do you know when his apt for the cath will be? did they move his next echo up or is the cath lab the next apt?
The next appointment is the cath. It will be scheduled for within the next month. After that, we will talk about the timing of 'interventions' (got to love the terminology).
Hi Shannon, Marin had that same procedure done where they cut out the ridge and widened the outflow. She has been doing amazing since that surgery almost one year ago. I'm sorry to hear about Wren. I know just how you feel. The progressiveness of Shones stinks, the inability to predict it as a parent is hard to deal with. I remember Marin's cardiologist telling us she was beginning to have problems and I was like, "what? she seems so fine." Can I add you on my blog? I keep meaning to check Wren's blog but have forgotten in awhile and now a lot has changed. I would love to keep up on his heart journey. Best of luck in the near future with your little guy
Hi Lindsey
Of course, I would be very happy if you follow our blog. I am always encouraged to learn of other families whose Kids have Shone's and are helping lead the way for us. I enjoyed reading about Marin's new "heart friend" a while back and feel the same way whenever I connect with another family.
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