Clinic went well - but we have a syndrome

Wren had his third cardiac clinic visit this morning. He had an echo, EKG, pressures, weights and growth and a consult. The quick summary is that it went well and he is stable.

The echo showed that his obstructions are similar to the last study. The arch has the same level of turbulence around the coarc site - not too severe. The other area of concern is the LVOT where there is a muscular ridge which is causing moderate turbulence in the ascending aorta from the aortic valve on up. The mitral valve is looking fine although the tech described it as slightly small and slightly prolapsed. It isn't causing turbulence. Neither valve has regurgitation or leaking.

The L-ventricle walls are still a little thick but its not enlarged and is pretty similar to last time. His pressures are normal - maybe a tiny bit high but not a cause for concern.

Last visit Dr Lewin said he would be aggressive in talking about OHS if the LVOT is causing him trouble. Today he said he would be conservative in suggesting it because he looks as if he is doing well and showing no clinical signs of stress which is encouraging.

During the consult I asked him about "Shones Syndrome" because I have been 'talking' to a number of parents online whose kids have a similar complex of left heart problems and have been diagnosed with Shones Syndrome. Dr Lewin said he would call Wren's condition Shones Syndrome but he doesn't find the diagnosis useful. Prognosis and treatment depends on the severity of each problem and how it responds to intervention. He says he prefers to focus on the area that needs attention. For now, that is the LVOT. In general, the literature on Shones is very dire - saying that it has very poor outcomes. Apparently the severity is mainly based on mitral valve involvment. In Wren's case, his mitral valve is working fine for now.

We were teased a number of times about Wren's weight and size. He has gone from 5.4kg a month ago to 6.8kg and is now in the 95th %ile for weight and 85%tile for length. His head is also growing (16") but we didn't chart it.

So, it was a good appointment overall.