18 mth ped visit - more information on bad news

Wren had his 18 month pediatrician appointment today along with his MMR shot. He was very brave and gave almost no complaint after the moment of the shot. His development is great along all normal measures:

Height: 33" 75th percentile.
Weight: 25 lbs 13oz - 5oth percentile!!!!
Head: 19 5/8" (85th percentile)

He has met or exceeded expectations for walking, running, climbing, speaking, understanding and communication in general. Dr L was very happy with him other than the sound of his heart which is "like a machine".

Uh oh.

And now the bad news... or rather more details on the bad news. At the appointment I received a copy of Dr Lewin's chart notes. These contain a lot more detail than the conversation we had at the appointment. They also make Wren's condition sound more serious.

Main BAD points:
* Wren has develop supravalvar aortic stenosis as well. The chart notes say that this requires investigation.
* He has stenosis in the aortic arch (again!) which was not mentioned.
* His aortic valve is thickened. This is a secondary aspect to aortic stenosis. Although he does not have regurgitation this is not a good development and may be due to the velocity of flow through the valve.
* His aortic valve is not only bicuspid but "domed". I have no idea what this observation means.
* Wren has mild "hypertension" (ie high blood pressure 118/67) in his right arm and slightly raised in right leg. They are delaying treatment (what? why?) until the cath is concluded and surgical path discussed).

There are GOOD points to:
* No regurgitation.
* Mitral valve behaving itself.
* LV normal size and only mild hypertrophy, good function.

Here is the concluding paragraph for those medical among you:

Wren is now 17 months old and carries a diagnosis of Shone's complex with multiple-level left heart pathology. This includes a bicuspid aortic valve, subaortic stenosis, coarctation of the aorta, an initial mitral valve abnormality and possible supravalve aortic stenosis. Wren is status post initial coarctation of the aorta repair with subsequent balloon aortic angioplasty as well as balloon aortic valvuloplasty. His echocardiogram today shows progressive left ventricular outflow tract obstruction which is now moderate to sever in nature. This progressive finding does place Wren at risk, and therefore I have recommended that we proceed with follow-up cardiac catheterization in order to better elucidate the level of maximal obstruction. My impression is that the subaortic process is quite significant but there also may be a major contribution from the aortic valve. I am hopeful that ballooning of the aortic valve will relieve the gradient such that we can avoid surgical intervention. However, I believe that we will be left with a significant subaortic process and there fore surgical intervention will be imminent. In addition, Wren has developed a supravalvar aortic process which also requires investigation. While Wren has only a 9mmHg blood pressure gradient [was 6mm before - my note] and his aortic arch appears only mildly turbulent by echo assessment, Wren has developed mild systemic hypertension in the right arm. I would suggest that we hold off on treating this until we have a better understanding of where we are heading regarding catheter-based intervention versus surgical.