Pediatric Heart Expert Forum
PAB for failure to thrive?
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PAB for failure to thrive?

My daughter is 5 months with a complicated medical history, including lobar holoprosencephaly, epilespy, ischemic stroke x2, diabetes inspidus, hemifacial microsomia w/ cleft lip and palate, heterotaxy (a, d, d, segmental anatomy) with polysplenia and associated intestinal malrotation.

She has been home from the hospital since September, where she has done excellent in all respects except weight gain (birth weight 2.99 kg, weight @ 5 months 3.5 kg).

They did a catherization which showed a qp to qs ratio of 8:1, with normal PA pressures. She was scheduled for immediate pulmonary artery banding. The surgeon says he placed the band as tight as it can go, and we are still getting a qp to qs ratio between 1.6-2.6:1, which I understand is still a moderate to large amount of shunting.

The current plan being proposed is to give her a month to see if she can grow now with the lowered qp to qs ratio. If not, then they are recommending a Glenn shunt be performed. This is all being done in the hopes of getting her big enough for a complex biventricular repair, which the surgeon says based on her cath data and her specific anatomy and the way her heart sits in her chest is not possible without her being much bigger: at least 6 kgs. If she can grow with the PA band, then we will leave that be for another 6 months or so and go for bivent. repair then.

My question for you: how realistic and possible is growth with a qp to qs ratio of 1.6 to 2.6 pulmonary blood flow?

How often is a Glen shunt used as an intermediary surgery to a biventricular repair? (her defects include C-AVC, d-tga, TAPVR with pulmonary venous return to right side and hepatic venous return to left, bilateral SVCs without bridging veins, interrupted IVC with left sided azygous return, pulmonary valve hypoplasia, mesocardia, RAA, mild common AV valve regurgitation, mild hypoplastic right ventricle...with a suggested modified Rastelli/Senning type double switch with aortic root translocation)
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As you might imagine, we cannot adequately state whether a specific procedure is indicated or not, without seeing all of the data  (not just select pieces of information).  However, one can say that it sounds like your child has had a significant improvement in the amount of left to right shunting after the pulmonary artery banding, and that means there is a better chance that weight gain, albeit slow, may occur.  The additional problem here is that patients with syndromes, like heterotaxy, may not grow well independent of their heart disease.  So the answer to your question about growth is that it may not be 100% heart related.  Doing a Glenn shunt (in her case, both right and left superior vena cavae to the lungs) would not change anything if the native pulmonary outflow tract and pulmonary artery were left as is...then you are just redirecting the same blood flow to the lungs by a different path and not changing the amount of left to right shunt. Doing a Glenn shunt requires that the pulmonary vascular resistance in the lungs be normal, and if the pulmonary artery pressure is completely normal, that may be the case.  But it sounds like there would have to be another step to that procedure, like oversewing the pulmonary artery or tightening the band further, in order to drop the amount of shunting into the lungs.  And that is a big step to take.  Maximizing the calories taken in each day is key to success here.  Clearly a complex operation will need to be done at some point, and getting her larger will be the goal if possible.  
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The Children’s Hospital of Philadelphia
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