We went to Seattle Children's Hospital for a cardiology appointment today. Wren had a chest X-ray, echo and consult.
I think that the appointment went ..... WELL!! The key words are "stable" and "hanging in there" and no procedures are needed for now. We are in a "wait and watch" stage with a follow-up in 6 months.
For those of you who don't know the background, Wren has Shone's syndrome with multiple problems in the left side of his heart, the side which pumps oxygenated blood to the body. The problems are mainly stenotic - which means that parts of the heart are too small which makes his left ventricle work harder to squeeze the required amount of blood through the obstructions.
The main areas we are watching are those repaired by Dr Hanley in November 2008: aortic valve and sub-aortic outflow tract. This appointment the gradient across these areas was stable. That is very good because in Shone's syndrome the blockages tend to be progressive but if Wren reaches a stable stage it could be a long while before he needs valve replacement surgery.
Joshua, ever the optimist, asked whether Wren will still need a valve replacement in the long term. I added, like by age 8? Josh felt that wasn't the long term and he meant by age 18. Dr Lewin said that "it depends" but he expected Wren to have a new valve by 18. This rather shocked me because I feel that we are forever teetering on the edge of valve replacement and cannot fathom the slightest chance Wren could make it to his teens before needing one. I thought Dr Lewin would laugh at the question.
After listening to Dr Lewin's explanation it seems that IF the AV area remains pretty stable we will now be watching the balance between the obstruction and the performance of Wren's left ventricle. The LV is now mildly thickened (not noticeably changed from before) but over time it is likely to thicken to maintain the work of forcing blood through the AV area. Right now, his LV is "hanging in there".
His mitral valve is also "hanging in there" with no appreciable increase in obstruction. The valve is abnormal but not stenotic and the regurgitation due to its abnormal function is only mild.
Wren's chest X-ray was clear (no more pneumonia) and Dr Lewin said it was not bacterial in the old X-ray and did not look severe.
The weights and heights were a bit flat - Wren was 13.2 kgs 4 months ago and is now 13.6 kgs (approx 30 lbs). However, I think that the last measure was a bit optimistic. He is 75th percentile for height and 50th percentile for weight. I need to make sure he eats but its ok otherwise and the lack of real weight gain may be due to a faulty weight last time and/or his 3 weeks of viral illness before school ended.
Overall, we are very relieved and quite happy. Wren is not healed but being stable is a pretty good second.