Yesterday, we had our second appointment with the heart doctors at Children’s Hospital. In brief, our daughter’s heart is still too small on the left side, but it did look better than the first appointment six weeks ago. Her aorta especially looked better. Her overall growth is good, 78% percentile. Of course, per Dave’s big head, her head is in the mid 80’s percentile-wise. We have another appointment, and possibly last prenatal appointment, in four weeks. Ultimately, nothing will be known or decided for certain until she is born.

It was nice that this appointment was only four hours instead of last time’s six hours. It was still full of all sorts of people, from the ultrasound tech, to the perinatal physician, pediatric cardiologist, social worker, and clinical care coordinator. Next time, we also get to meet with the NICU and the cardiac surgeons. Those are the people who have the most say over our daughter’s care.

The pediatric cardiologist was the same one as last time. Her reaction was extremely encouraging about how much improvement she had seen. The mitral valve is still probably the worst part of the heart. She gave us a new possible diagnosis to look up: Shone’s Syndrome.

Children’s Hospital has not received anything from Boston. We gave them the verbal report that Boston gave us, and Children’s agreed that she could have a two ventricle heart. They even agreed that the possibility existed that she might not even need the scary side effects drug PGE, but they cautioned that they like to be conservative, start the drug, and then stop it if not necessary.

Dave and I came with a whole page length of typed questions. Many of the questions involved the plan from here on out. They took the time to answer all our questions. It was very encouraging. In general, I am very happy that they had no problem with us staying with the midwives from the Minnesota Birth Center for both the weekly follow-ups starting at 32 weeks, and for delivery. We still have a few points of contention, but the conversation has been started. They have been warned what I want should medical conditions allow, and that I can be stubborn.

Dave will share his thoughts a bit later.

About a week ago, I contacted Boston Children’s Hospital for a second opinion. They are the world leader in hypoplastic left heart syndrome (HLHS), and do many things that other places don’t, such as fetal surgery. 

Monday, they finally received all of our medical records. A doctor, Dr. Friedman, reviewed the results and actually had better news.

By the way, this is a long post. If you don’t feel like reading it all, skip down to “In Summary,” near the end. 

Boston does not believe that the diagnosis of HLHS applies. Yes, they see that the left side is smaller, but Dr. Friedman thinks that it will very likely be big enough to pump blood to the body. He does not think that the series of 3 surgeries will be needed.

They also did not see any aortic stenosis. That means that no fetal intervention is recommended. So, I will not be going to Boston for fetal surgery.

The diagnosis that Boston believes is most accurate is Coarctation of the Aorta (COA), or a narrowing of the aorta. This is, in general, less serious than HLHS. It will still require repair, but is much simpler than the series of 3 surgeries for HLHS. Plus, COA repair has a much higher survival rate than the HLHS 3 surgeries.

Boston believes that our local Children’s Hospital should be able to handle this. Here is the information page about COA from Children’s Minnesota. Boston wants us to mention this diagnosis to our local care team and see what they have to say. Boston reconfirmed that I need to deliver our daughter where we are planning on having her surgery. Right now, they see no reason why we should have to go to Boston. However, they would like us to call them back after our next follow-up fetal echo cardiogram on January 17th.

In Summary:

In short, we received good news this week. Boston believes it likely that our daughter will have two pumping chambers. Instead of the diagnosis of HLHS, they think coarctation of the aorta (COA), or narrowing of the aorta, is more applicable. No fetal intervention or surgery is recommended at this time. In fact, they do not see the need for us to go to Boston at all. However, we are still planning on a heart repair once she is born, with extended NICU time.

We both feel some relief, and slept better last night. Our daughter is not out of the woods. We still have a long, hard journey ahead of us. But at least there is a bit more hope. We still welcome thoughts and prayers.

Please read Dave’s Journal Entry first. This entry assumes that you have already read his.

Thanks for coming to our website. If you know me, you probably know that I don’t share personal things easily. This situation is no exception. Please know that I welcome any questions you have, at any time. Even if I do not offer the information, I am willing to answer questions. Don’t hesitate to ask. Dave and I are slowly mastering the skill of talking about this without tearing up, and they say practice makes perfect.

I attached a picture of a normal heart vs. a hypoplastic left heart syndrome heart. It is from the CDC article Dave linked to. Our daughter’s heart is not quite as bad as the one in the image, but it gives the general idea.

Right now, we are waiting to hear back from some questions we asked on Monday. There is a small chance of fetal surgery, possibly at Boston Children’s Hospital. We should know more in a week. Otherwise, it is just increased monitoring of this pregnancy, and waiting until she is born.

That moment, minutes after she is born, that they take her away will certainly be a sad one. We welcome any thoughts and prayers.

It’s been a wild week. Last Thursday Audrey and I learned that our little one likely has a Congenital Heart Defect. Specifically, she has an issue called Hypoplastic Left Heart Syndrom, or HLHS. HLHS is a situation where the left side of the heart is underdeveloped and can’t function properly. There is no danger right now while our little one is inside but when she starts to breathe on her own, if untreated it would likely be fatal in several days. 15-20 years ago, it would be fatal. Luckily, there are some risky, but apparently effective procedures that can restore a mostly normal way of life.

So what happened? Audrey’s now into her 22nd week of the pregnancy. At the 20 week Ultrasound, the Dr from the awesome Minnesota Birth Center thought he detected a 2-vessel umbilical cord and possibly a smaller left ventricle in the heart. While the Dr was formerly a Perinatal Physician, he hadn’t been in that field for a few years and recommended a second look. Audrey and I had to wait 1.5 weeks but we got in to see the staff at the Minnesota Perinatal Physicians at Children’s Hospital in Minneapolis. After a long series of detailed ultrasounds, we talked to a Perinatal Physician, a Neonatal Cardiologist, a Genetic Counselor and a Care Coordinator. They gave us the diagnosis of HLHS. 

Our diagnosis is currently borderline so we’re hopeful. The current, worst case process for dealing with this will involve a series of 3 open heart surgeries. One immediately a few days after birth. Another around 4-6 months and another one somewhere at 18 months to 5 years. The procedures basically convert the heart from a 4-chamber organ to a 2-chamber organ.

We’re scared, unsure and sad, but also extremely happy to know about this now, before our little one is born rather than learning at birth and having to deal with all of this all at once. 

We’ll share more soon but wanted to share some basics. 

If you want to learn more, here’s a link to an article from the CDC with some Facts about HLHS to start you down the rabbit hole.

In short, Audrey is healthy and safe. Little baby Royer is healthy and safe while inside mom. This doesn’t affect the way we handle the pregnancy right now other than additional monitoring.

Our 20 week ultrasound was not a happy day full of pretty pictures, but instead the start of a long journey to address a critical congenital heart defect. Follow along by reading our Journal.

These journal entries make the most sense if you read them all, from oldest to newest. If you haven’t already, we encourage you to read from the first entry, “A Turn of Events.”