Friday, September 26, 2008

For Shones Complex - multiple OHS are better than transplant

I subscribe to some citation search tools which alert me when an article is published on Shone's Complex. This cheery little piece came through my inbox this morning.

Outcomes of Reparative and Transplantation Strategies for Multilevel Left Heart Obstructions With Mitral Stenosis
Malhotra, S. P., Lacour-Gayet, F., Campbell, D. N., Miyamoto, S., Clarke, D. R., Dines, M. L., Ivy, D. D., Mitchell, M. B.

Apparently, from a study of 43 children with Shone's Complex - some of whom received a heart transplant while others had staged heart surgeries for mitral stenosis and LVOT obstructions, they learned:

Results: There was one in-hospital death (2.5%) and six late deaths (14.2%). Actuarial 5- and 10-year survival for staged surgical and transplantation was 88% vs 61.3% and 83.1% vs 61.3% (p = 0.035). At a mean follow-up of 7.9 years, freedom from mitral reoperation was 83.3% and freedom from reoperation for subaortic stenosis was 78.0%. Wait-list mortality was 13.3% (2 of 13). Wait-list time exceeding 90 days was an incremental risk factor for death after transplantation (p = 0.005).

Conclusions: Despite the challenges of a reparative strategy for Shone's complex, favorable survival and durability outcomes can be expected. Heart transplantation, although avoiding the pitfalls of staged repair, confers increased risks from ongoing physiologic derangements due to uncorrected left heart inflow and outflow obstructions during the wait for donor heart availability.

This means that on average without differentiating for severity of the presentation 88% of kids who had the kind of surgeries Wren faces lived for 5 years and 83.1% were alive after 10 years.

The 5 year survival rate for those who received a transplant was 63.1 % and no further children died by 10 years out. So, the initial risk was quite a bit higher for the transplant but it was stable once the surgery was a success. Of course, the longer term issues with transplant are murky.

At least, in Wren's case it seems that the risk of staged surgeries is preferable and since he has low level of mitral valve problem right now, that he has at least an 83.1% chance of being alive in 10 years.

Another little nugget was that 78% of kids who had LVOT repairs did not require reoperation within 7.9 years. That is not terribly encouraging since Wren has a relatively complex form of LVOT obstruction. Still, it does not contain enough detail [presentation, type of obstruction, surgical repair done, residual gradient, age etc] to draw meaningful conclusions.

Still waiting to hear from our second opinion cardiologist, Dr Krabill. I feel her delay is encouraging - it means she doens't just agree with Dr Lewin... she is doing her own research. I need that from someone else.

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