This Heart Broken

We will be meeting with the chief of the heart transplant program at Children’s Hospital this Wednesday, May 31st.

Our cardiologist called this evening (finally) and let us know what the team at Children’s had decided. He met with approximately 10 cardiologists, the chief of transplant surgery, several surgeons and some electrophysiologists. They all agreed she needs a pacemaker because her heart rhythm is not regular. The surgeons were very nervous about attempting any surgeries with a pacemaker coupled with her complex heart problems. A couple of the cardiologists felt that it could be done but the surgeons’ issue was less about surviving the surgery itself and more about surviving after the surgery. The team came up with three different surgery options immediately after birth but none of them would allow her to live much more than a few months. Essentially, these surgery options only get her out of the neonatal period before attempting a heart transplant; they would potentially allow her to go home until a heart became available for transplant. However, by doing an initial surgery before transplant there are some serious drawbacks. First of all, these surgeries carry a pretty high mortality risk especially with a pacemaker implantation. Secondly, by waiting until she’s 4-6 months old for a transplant, she would lose the advantage of a lowered immune system at birth. A newborn has a very low immune system but when you pump someone else’s blood into them (like during a surgery) their immune system revs up. One of the advantages of infant heart transplant over one later in life is an infant’s immune system doesn’t know to reject the heart; that doesn’t last forever but it can help deter early rejection. In that way it is better to do transplant surgery on a newborn rather than on a 4 month old baby.

There are a few ways we could pursue a transplant for her at birth. We could put her on a donor list prenatally (at about 36 weeks gestation) but that would require me being willing to have a c-section as soon as the heart became available. We could also put her on the list the day she is born or we could wait until 10-14 days after birth when they have finished doing all heart evaluations on her.

Dr. Lewin is arranging for us to meet with the chief of heart transplantation at Children’s Hospital so we can have some questions answered. It is VERY difficult to find any information on infant heart transplants, day-to-day life and long term results since this is a relatively new procedure.

Her next fetal echo will be June 8th. Right now we have to decide if we want to pursue a heart transplant, how much monitoring we wish to do of her condition before she’s due and how much monitoring during the birth we would like.

We went to University of Washington Hospital today to do an ultrasound, an echo and to meet with the perinatology and neonatology specialists. Here is a rundown of what they found. Her size is okay and her amniotic fluid level is fine. The small amount of fluid around her heart hasn’t changed at all but it appears that her valve leakage may have progressed. It was hard for our cardiologist to tell because he was using a different machine than he’s used on her previous echos. He also detected that the valve to her pulmonary artery has a little leakage. The heart is squeezing well, and there is plenty of blood flow to the body. Her heart rhythm is a problem again. She now has what Dr. Lewin calls a “second-degree heart block. This means that there is some communication between the upper chambers and the lower chamber of her heart but there is nothing to stand in the way of losing that communication. It is not considered a benign heart rhythm and Zoe would need a pacemaker with this heart rhythm. Dr. Lewin and the perinatologist sat with us and told us we were back in the situation again where none of our options are very good. If we ask the surgeons to do her initial surgery (the
Norwood) she would have to have a pacemaker put in and it would greatly reduce her chances of surviving the surgery. If she lived and the surgery was successful she would have massive problems over time because we would be asking her one ventricle to do the work of all four chambers her heart should have. This problem rolls over into the next two surgeries and Dr. Lewin was very clear she very well may not survive any surgeries past the first one. He also told us her second surgery (the
Glenn) probably wouldn’t work anyway because of her valve leakage.

Our options include: not pursuing surgery; trying the first surgery with the pacemaker and then either trying the second surgery or attempting a transplant at 4-6months or attempting a transplant at birth. At this point, Dr. Lewin will be presenting our case to the surgeons, the specialists that deal with the electric issues in hearts and the other cardiologists at Children’s Hospital. After this meeting he will call us and let us know what their recommendations are. They have asked us to decide, in the next three weeks, how aggressively we would like to deal with her heart issue. This will make a huge difference in how she is monitored during labor and what interventions will be given immediately after birth. I asked Dr. Lewin if this heart rhythm problem could go away and he told us “no”.