Adriana, born 2005During my amnio, it was discovered my baby had an omphalocele - whereby some of the organs develop in a little sac outside the body and do not retreat back inside as they should. We were warned at that time this could be a sign of a congenital heart defect or a chromosomal abnormality. I waited twelve agonizing days for the results. Thank God there were no chromosomal abnormalities! It would be another two months or so before a fetal echo could be done. First one wasn't too clear but appeared to reveal Transposition of the Great Arteries and a Ventricular Septal Defect. Once she was born, Double Outlet Right Ventricle was added to the mix. We were told any attempt to repair her VSD would be fatal. The VSD would be opened further and she would have a three chamber heart.
Her omphalocele surgery was first. On June 2, 2005, at two hours of life and weighing 5 pounds and 3 ounces, she was in the OR having a silo put over her organs. This silo would be "cranked"down twice a day for five days until her organs (bowel and liver) retreated inside enough for the abdominal wall to be surgically closed. She was expected to have a BT Shunt put in three days later but no one was comfortable with her going back into the OR so soon. So, she was kept on prostaglandin to keep her PDA open until the shunt could be done.
Because of scheduling problems, I had Adriana moved to another hospital for surgery which was now postponed for an MRI would confirmed stenosis of the LPA. They were concerned that if the PDA closed, it would close off the LPA. The surgeon had to decide on the fly whether to just do the shunt or to place a stent in the LPA. Thankfully, he chose the BT Shunt and the PDA closed fine.
We noticed a difference being at this hospital, though. They confirmed for us through many echocardiograms that she was not TGA, nor DORV, so they swore they were 100% sure her VSD could be closed and she would have a four chamber heart. She's now classified as Tetralogy of Fallot with Pulmonary Atresia.
Hearing this, I had both hospitals contact each other and now both confirm she doesn't have TGA but neither is sure any longer about her having DORV which will determined the 3 or 4 chamber heart. Adriana is scheduled for a catheterization next year to finally confirm a diagnosis. We will not know until then what the future holds for our little baby girl. It is expected her open heart surgery will be done 3-4 weeks after that.
She recently received a G-tube to help her gain weight as the more she weighs at the time of surgery, the safer the surgery will be. At twenty one weeks old, she weighs 9 pounds, 6 ounces. I will add to her story after the cath and or surgery has been done. Please keep praying.
Story by Adriana's mom, Mary - New York
Andrew, born 2004
Asher, born 2010