My 14 month old was born with "physiologically corrected" TGA/heterotaxy--- her ventricles were in the right position, put she had D-tga, TAPVR, interrupted IVC with azygous continuation to the left SVC, bilateral SVCs, and mildly left dominant AV canal defect (57% of AV valve surface area to the LV), complex hepatic return and severe sub, supra and valvular PS. She had a Nikaidoh procedure with an additional baffling of the neo-aorta to the LV, plus RV-to-PA conduit, her pulmonary veins moved from the RA to LA, and baffles of her left SVC and hepatic veins to RA, and an AV canal repair.
Her cardiologist mentioned in passing that a very small percentage of children who go from having a systemic RV to systemic LV a la "double switch"-esque procedure she just had can develop LV failure over time.
The only information I can find on that phenomenon references those with isolated l-tga or those with discordance anomalies who are older and whose LV required "training" to handle systemic pressures. My daughter required a PA band in order to stop pulmonary overcirculation, and because of the AV canal defect, my understandig is that both ventricles were functioning at systemic pressure?
If that is the case, then isn't it reasonable to presume that LV failure wouldn't really be a potential complication that applies to cases where the LV has always been subjected to systemic pressures?
Also, PVS was also mentioned as a small risk, because they had to move her pulmonary veins. However, they did a "sutureless" move of her veins, and they have always been unobstructed. She only has two pulmonary veins--- a right and a left (each upper and lower vein forms a confluence before entering the atrium). Doesn't a sutureless move of the veins reduce the likelihood of developing any type of obstruction?
I will be discussing this more in depth with her cardiologist at our next appointment in regards to her specific anatomy, but perhaps you could give general risks? TIA
I'm not sure we know exactly why these patients have LV failure, per se. I think that one thing that we do see is that, despite having had a pulmonary artery band, the left ventricle can "decondition" and lose its ability to handle normal pressures. Also, considering that the heart is grossly structurally abnormal, there may also be some cellular or DNA signaling that may cause it to just not behave normally over time. Certainly, having undergone a prolonged cardiopulmonary bypass (at least longer than that of a typical VSD repair) may not be a good thing for the heart to have had to endure, and may potentially adversely influence its long term abilities. It may be that the baffling of the atria may change the architecture of the heart, although this is less likely. Finally, there may be other factors of which we just are not aware that may be to the detriment of the heart of the long haul. The bottom line is that, not unexpectedly, your daughter will need continued, lifelong surveillance for her heart, first with a pediatric cardiologist, and eventually with an adult congenital heart disease specialist.
With regard to the "sutureless" repair of pulmonary veins, yes, it is supposed to reduce the incidence and occurrence of pulmonary venous stenosis. However, it has still happened in some cases, so it's not totally dropping the risk to zero. I would expect to see it sooner than later, though, but this will be observed, as well.
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