I received an answer to my original question, but realize I left out some info. My unborn son has been diagnosed w/ pulmonary atresia with hypoplastic right heart, as well as tricuspid atresia. The answer to my last question explained the possibility of perforating the atresic pulmonary valve plate to make the heart a two-ventricle heart. The option given by the doctors was for the shunt to replace the ductus arteriosus, which will require other surgeries as he grows. The explanation of the tricuspid atresia was that there is a back-flow into the tricuspid valve. Also that the right ventricle is smaller than it should be. Also now, they have discover that the baby has a dysrythmia w/ PAC. Doctors have already decided that the ductus arteriosus will be kept open using prostaglandin & they want to perform the surgery within the week of his birth.
My next question is, With all of these things being present, is the Shunt procedure more than likely the best option for my son? Also, I am currently ( @ 7 1/2 mths pregnant) at risk of going into preterm labor, and have already dialated one centimeter, with the fetus currently weighing 2lbs10oz. What are the risks and are they greater if the infant is born under normal birth weight of at least 5lbs? Also in light of all information given is it more than likely that these things will play a role in the option of perforating the atresic valve plate or going ahead with the shunt operation.
If the right ventricle is severely hypoplastic, then your son will have to undergo placement of a modified Blalock-Taussig shunt to allow blood to get to the lungs. He will be treated as if he has what is referred to as single ventricle physiology, which means that he only has one functioning ventricle. He will eventually require conversion of the initial shunt to a Glenn anastomosis, which takes down the B-T shunt and connects the venous blood return from the head and neck directly to the pulmonary arteries. Later, a third surgery will involve the Fontan anastomosis, in which his lower body venous return will be connected directly to the pulmonary arteries.
In the end, based on what you are telling me (and in the absence of evaluating your son), it appears that a shunt OR cardiac transplantation would be the only two choices. Do realize that the coronary arteries occasionally can be dependent upon the elevated right ventricular pressures and can cause the heart to fail over time if this is not able to be remedied. In these cases, cardiac transplantation is the only option. Finally, a smaller birth weight certainly does increase the risks of surgical outcomes, including putting in a shunt that is too big, having him outgrow the shunt more quickly, kinking the pulmonary arteries, and damaging the lungs. Hopefully your son will make it to term to reduce this risk.
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