Avery's Heart

My daughter, Avery Elizabeth Bell, was born on June 9, 2009 via C-Section. Prior to birth, we found out that Avery had Turner's Syndrome (see Turner's Syndrome post for more information) after having an amniocentesis due to significant medical findings. The "medical findings" I mentioned above was a large cystic hygroma that was covering her entire neck and back in utero. The hygroma was supposed to continue growing, taking the blood supply from her body, but instead, began to miraculously shrink. I feel this was as a result of fervent prayers from all across the country. She did have around a 1% chance to survive when we first found out, so doctors began to care for me as high-risk, and I waited to see if there would be a heartbeat at every 2 week appointment. When things began to turn around for the better, I was sent to a cardiologist for fetal echocardiograms twice to search for heart defects associated with Turner's Syndrome. The cardiologist was unable to tell whether or not our baby would have the common defects, so at delivery, we did a C-Section birth to ensure all needed medical staff would be available.
I say I was lucky because lots of times heart defects and others issues associated with Turner's Syndrome are unknown until after birth when things go wrong quickly. Thankfully, we scheduled Avery's birth at a point when Dr. Richard Jonas of Washington D.C., would be in town at University Medical Center just in case she was born with any CHDs. Avery was born around 7:30 in the morning, and I was so relieved to hear that new baby scream. I think it is a surreal moment for every parent, but even more so for me because I wasn't sure if she'd live for the longest time. Doctors whisked Avery away, and the tests revealed she did have an aortic coarctation, so she was transported to UMC's NICU to be put on Prostaglandin (forgive me for medical term. spelling) to keep her ductus open until surgery. The medication (which I was unaware of until sometime later) caused her to have apnea (which is a side effect) several times, and Avery began to code. How my husband must have felt, seeing her stop breathing with people rushing to her aid, as his wife was at another hospital. Avery began to do better on the meds, and besides being somewhat jaundice, she was doing ok. She went in for surgery the day she turned 1 week old at 4 lbs and 15 ozs. The risk wasn't near as bad as I thought it would be since she also had some kidney issues- around 90%. Dr. Jonas performed an extended end to end procedure through closed heart surgery which went very well. Avery also had a bicuspid aortic valve, which ended up being more monocuspid once her next surgeon got into surgery.


She was monitored monthly by our local cardiologist, and had two catheterizations a few months after her first surgery. The first cath was to balloon her valve and the coarc which was repaired in surgery. The second cath was to take accurate measurements. Avery's echos were reading very poorly, and she was doing too well clinically to have those awful numbers. We knew she has a significant leak in her valve, but she had no trouble breathing and seemed to be doing fine with good circulation. After our doctor took the accurate measurements, he told us we could then hold off on her next surgery for a while. Unfortunately, we were unable to hold off as long as we thought. Avery went in for her next check-up and we were told she needed surgery at that point. She would only get worse, and the chances of waiting were too risky. We were then sent to New York City, Morgan Stanley Children's Hospital, so Dr. Emile Bacha (see information about him and Dr. Jonas on this blog) could perform an open-heart valve replacement surgery with the Ross Procedure. This surgery was also very successful. Avery now has a cadavor valve where her pulmonary valve was, and the pulmonary valve is now her aortic valve. She does have some leakage that is (for now) insignificant, but she shouldn't have to have another surgery for some time now. She may have to enter the cath lab for further balloons, etc., but nothing too serious in the very near future...hopefully. Since her last surgery, her cardiologist couldn't be happier with her heart, and says it is functioning beautifully. We were given a 3 month break from appointments, and go back to see him for an echo next week.
Avery has been gaining weight, and hasn't missed a milestone even with all of her complications. She is small in stature, and probably looks about 9 months rather than 13 months. The first thing people say when they see her is..."How old is she?" and "Gosh, she is so small!" She should be walking soon, and is an extremely active little red-head. She is tough, and she has the scars to prove it!!